Practice nursing care for Clients

 

Practice nursing care for Clients

with Disorders of Hemostasis

 

 

 

The hematologic system is composed of the blood, blood cells, lymph, and organs concerned with blood formation or blood storage. Because all systems depend on circulation and lymph flow, any problem of the hematologic system has widespread consequences for health and well-being. This chapter review the normal physiology of the hematologic system and the assessment of hematologic status.

ANATOMY AND PHYSIOLOGY REVIEW

Bone Marrow. Bone marrow is the blood-forming (hematopoietic) organ. It produces most of the cellular elements of the blood, including red blood cells (RBCs, erythrocytes), white blood cells (WBCs), and platelets. Bone marrow also is involved in some aspects of the immune response. Each day, the bone marrow in a healthy adult produces and releases approximately 2.5 billion RBCs, 2.5 billion platelets, and 1 billion white blood cells (leukocytes) per kilogram of body weight. In the fetus, blood components are formed in the liver and spleen and, by the last trimester, in the bone marrow. At birth, blood-producing marrow is present in every bone. The flat bones (sternum, skull, pelvic and shoulder girdles) contain active blood-producing marrow throughout life. As a person ages, the amount of functional bone marrow decreases in the long bones and in small, irregularly shaped bones. By age 18 blood production is limited to the ends of the long bones. During adulthood, fatty tissue replaces inactive bone marrow. In older adults, the proportion of fatty marrow increases to approximately one half of the marrow found in the sternum and ribs, and only a relatively small portion of the remaining marrow continues active blood production.

The bone marrow produces all blood cells, which initially start out as stem cells. Bone marrow contains pluripotent stem cells. Pluripotent stem cells are immature and undifferentiated cells that are capable of maturing into any one of several types of blood cells: RBCs, WBCs, or platelets, depending on the body’s needs (Figure 39-1).

 

 

The next stage in cell development is the committed stem cell (also called the precursor cell or unipotent stem cell). A committed stem cell has one specific maturational pathway and matures or differentiates into only one cell type. Committed stem cells actively divide but require the presence of a specific growth factor for further development and maturation. For example,erythropoietin is a growth factor made in the kidneys that is specific for the red blood cell line. Many different growth factors influence the maturation of white blood cells and platelets.

 

Blood Components

 

Blood is composed of plasma and cells. Plasma, part of the extracellular fluid of the body, is similar to the interstitial fluid found between tissue cells. Plasma, however, contains about three to four times more protein than does interstitial fluid. There are three major types of plasma proteins: albumin, globulins, and fibrinogen. The primary function of albumin is to increase the osmotic pressure of the blood, which prevents plasma from leaking into the tissues. Globulins perform many functions, such as transporting other substances and protecting the body against infection. Globulins are also the main component of antibodies. Fibrinogen is a protein molecule that can be activated to form fibrin. Individual molecules of fibrin assemble to form large structures important in the blood-clotting process.

The cells of the blood include RBCs, WBCs, and platelets. These blood cells differ in structure, site of maturation, and function.

 

RED BLOOD CELLS (ERYTHROCYTES)

 

Red blood cells (RBCs), or erythrocytes, make up the largest proportion of blood cells. Mature RBCs have no nucleus and have a biconcave disk shape. Together with a flexible membrane, this feature allows RBCs to change their shape without breaking as they pass through narrow, winding capillaries. The number of RBCs a person has varies according to gender, age, and general health, but the normal range is from 4.4 to 5.5 million/mm3. As shown in Figures 39-1 and 39-2, RBCs start out as pluripotent stem cells, enter the myeloid pathway, and progress in stages to the mature RBC (the erythrocyte).

 Healthy, mature RBCs have a life span of approximately 120 days after being released into the blood from the bone marrow. As RBCs age, their membranes become more fragile. These old cells are trapped and destroyed by fixed macrophages in the tissues, spleen, and liver. Some parts of destroyed RBCs (e.g., iron) are recycled and used in the formation of new RBCs. RBCs are responsible for producing hemoglobin (Hgb). Each normal, mature RBC contains many thousands of hemoglobin molecules (Guyton & Hall, 2000). The heme part of each hemoglobin molecule requires a molecule of iron. Only when the heme molecule is complete with iron can it transport up to four molecules of oxygen.Therefore iron is a critical component of hemoglobin.

The globin portion of the hemoglobin molecule carries carbon dioxide. RBCs also serve as a buffer and help maintain acid-base balance. The most important feature of the hemoglobin molecule is its ability to combine loosely with oxygen. With only a small drop in oxygen concentration at the tissue level, a greater increase in the transfer of oxygen from hemoglobin to the tissues occurs. This transfer is also known as oxygen dissociation. Some pathologic conditions change the speed and amount of oxygen released to the tissues.

The total number of RBCs is carefully controlled through erythropoiesis (selective maturation of stem cells into mature erythrocytes). This process ensures that enough RBCs are present for good oxygenation without having an excess concentration, which could “thicken” the blood and slow its flow. The trigger for control of erythropoiesis is tissue oxygenation. The kidney produces the RBC growth factor erythropoietin at the same rate as RBC destruction to maintain a constant normal level of circulating RBCs. When tissue oxygenation is less thaormal (hypoxia), the kidney increases the production and release of erythropoietin. This growth factor stimulates the bone marrow to increase RBC production. When tissue oxygenation is excessive, the kidney decreases the production erythropoietin, inhibiting the production of RBCs. Synthetic erythropoietin (Procrit, Epogen, EPO) is now available and appears to have the same effect on bone marrow as naturally occurring erythropoietin.

Many substances are needed to form hemoglobin and RBCs, including iron, vitamin B12, folic acid, copper, pyridoxine, cobalt, and nickel. A lack of any of these substances can lead to anemia. Anemia is the result of any condition in which either the function or the number of erythrocytes is inadequate to meet tissue oxygen demands.

 

WHITE BLOOD CELLS (LEUKOCYTES)

 

White blood cells (WBCs), or leukocytes, are the second category of blood cells. There are many types of leukocytes, and each type performs at least one specific action critical to inflammation or immunity (Table 39-1).

 

 

Most WBCs are formed in the bone marrow and are considered part of the hematopoietic system. A detailed discussion of leukocyte anatomy and function is presented in Chapter 20, because these cells provide immunity and protect against the effects of invasion, infection, and injury.

 

PLATELETS

 

 

 

Platelets are the third type of blood cells. They are the smallest of the blood cells and are formed as fragments of a giant precursor cell in the bone marrow, the megakaryocyte. Figure 39-1 shows the overall developmental pathway of blood cells, and Figure 39-3 shows specific platelet development.

Platelets stick to injured blood vessel walls and form platelet plugs that can stop the flow of blood from the injured site. They also produce substances important to coagulation. Platelets maintain blood vessel integrity by beginning the repair of damage to small blood vessels. They perform most of their functions by aggregation (clumping). Platelet production in the bone marrow also is precisely controlled by growth factors (thrombopoietin). After platelets leave the bone marrow, they are taken up by the spleen for storage and are released slowly according to the body’s needs. Normally, 80% of platelets circulate and 20% are stored in the spleen. Each platelet has a life span of 1 to 2 weeks, after which it is gradually used up or destroyed during normal clotting activities.

Accessory Organs of Hematopoiesis The spleen and liver are important accessory organs of the hematopoietic system. They help regulate the maturation of blood cells to help maintain hematologic homeostasis.

 SPLEEN

The spleen is located under the diaphragm to the left of the stomach. It contains three types of tissue—white pulp, red pulp, and marginal pulp—all of which help to balance blood cell production with blood cell destruction and assist with immunologic defensive mechanisms. White pulp is filled with lymphocytes and macrophages. As whole blood circulates and filters through the white pulp, unwanted cells (e.g., bacteria and old RBCs) are removed. Red pulp is composed of vascular enlargements (sinuses) that are storage sites for RBCs and platelets. Marginal pulp contains the ends of many arteries and other blood vessels. During blood formation, the spleen destroys old or imperfect RBCs, assists in iron metabolism by breaking down the hemoglobin released from these destroyed cells, stores platelets, and filters antigens. A client who has undergone a splenectomy experiences an impairment of some immune functions. Thus after a splenectomy, the body is not efficient at disposing of many bloodborne pathogenic microorganisms and is at a greatly increased risk for infection and sepsis (Workman, 1998).

 

LIVER

 

The liver is important for normal erythropoiesis and is the primary production site for prothrombin and most of the bloodclotting factors. In addition, proper liver function and bile production are critical to the formation of vitamin K in the intestinal tract. (Vitamin K is essential in the formation of blood-clotting factors VII, IX, and X and prothrombin.) Large quantities of whole blood and blood cells can be stored in the liver. The liver also converts bilirubin (one end-product of hemoglobin breakdown) to bile and stores extra iron within a storage protein called ferritin. Small amounts of erythropoietin are produced in the liver.

 

Hemostasis/Blood Clotting 

 

In hemostasis, selective localized blood clotting occurs in damaged blood vessels while blood circulation to all other areas is maintained. Hemostasis is a complex process that balances the production of clotting and dissolving factors. It begins with the formation of a platelet plug and continues with a series of events that eventually cause the formation of a fibrin clot. Intrinsic and extrinsic factors are involved in fibrin clot formation and blood coagulation. Three sequential processes result in blood clotting: platelet aggregation with formation of a platelet plug, the blood-clotting cascade, and the formation of a complete fibrin clot.

 

PLATELET AGGREGATION

 

 

 

The formation of a platelet plug by causing platelets to aggregate (clump) together is a key requirement for blood clotting. Platelets normally circulate as individual cell-like structures. They are not attracted to each other and do not clump together until activated or until the presence of other substances causes platelet membranes to become sticky, allowing aggregation to occur. When platelets become activated and aggregate, they form large, semisolid plugs within the lumens and walls of blood vessels and disrupt blood flow. These platelet plugs are not clots and cannot provide complete hemostasis. Some substances that cause platelets to aggregate include adenosine diphosphate (ADP), calcium, thromboxane A2, and collagen. Platelets themselves can be stimulated to secrete some of these substances, whereas other substances causing platelet aggregation are exogenous. Formation of a platelet plug starts the cascade reaction that ultimately causes blood coagulation to occur through formation of a fibrin clot.

 

THE BLOOD-CLOTTING CASCADE

 

The blood-clotting cascade is triggered by the formation of a platelet plug. Platelet plug formation results from intrinsic or extrinsic factors. The beginning of this cascade is rapidly amplified or enhanced, with the final result much larger than the triggering event. Cascades work like a landslide. A few small pebbles rolling down a steep hillside can dislodge large rocks and pieces of soil, causing a final enormous movement of earth. As with landslides, cascade reactions are hard to stop once set into motion. Intrinsic Factors Intrinsic factors are problems or substances directly in the blood itself that first make platelets aggregate and then proceed to activate the blood-clotting cascade (Figure 39-4).

 

 Intrinsic events that stimulate platelet aggregation include antigen- antibody reactions, circulating debris, prolonged venous stasis, and bacterial endotoxins. Continuation of the cascade to the point of fibrin clot formation depends on the presence of sufficient amounts of all the various clotting factors and cofactors (Table 39-2).

 

Extrinsic Factors Platelet plugs can begin to form as a result of changes in the blood vessels rather than changes in the blood. When platelet plugs form in response to blood vessel changes, the response is said to be caused by extrinsic factors (extrinsic to the blood). The most common extrinsic events are trauma to tissues and damage to blood vessels, which exposes the platelets to collagen and stimulates aggregation. The platelet plug is formed within seconds of the trauma. The blood-clotting cascade is started sooner by the extrinsic pathway than by the intrinsic pathway because some of the steps of the intrinsic pathway are bypassed. Whether the platelet plugs were formed because of abnormal blood (intrinsic factors) or by exposure of substances from damaged blood vessels (extrinsic factors), the end result of the cascade is the same: formation of a fibrin clot and coagulation. The many steps of the cascade between the formation of a platelet plug and the formation of a fibrin clot are dependent on the presence of specific clotting factors, calcium, and more platelets. Clotting factors (see Table 39-2) are actually inactive proteins that become activated in a sequence to activate fibrinogen into fibrin. At each step, the activated protein from the previous step allows activation of the next protein. The last two steps in the cascade are the activation of thrombin from prothrombin and the conversion (by thrombin) of fibrinogen into fibrin. Only fibrin molecules can begin the formation of a true clot.

 

FIBRIN CLOT FORMATION

 

Fibrinogen is a large, inactive protein molecule made in the liver and secreted into the blood. Thrombin, an enzyme, removes the end portions of fibrinogen and converts it to the active fibrin molecule. Individual fibrin molecules link together to form fibrin threads. The fibrin threads make a lattice-like meshwork that forms the base of a blood clot (Figure 39-5).

 

 

After the fibrin mesh is formed, a stabilizing factor (clotting factor XIII) tightens up the mesh, making it more dense. Additional platelets stick to the threads of the mesh and attract other blood cells and proteins to form an actual blood clot. As this clot retracts, the serum (plasma without the clotting factors) is excreted, and clot formation is complete.

 

FIBRINOLYSIS

 

Because blood coagulation occurs through a rapid cascade process, in theory it keeps forming fibrin clots whenever the cascade is set into motion until all blood throughout the entire body has coagulated. Such widespread coagulation is not compatible with life. Therefore, when the blood-clotting cascade is started, counterclotting or anticoagulant forces are also started to limit clot formation to only damaged areas; normal blood flow is maintained everywhere else. When blood clotting and anticlotting actions are appropriately balanced, coagulation occurs only where needed, and normal circulation is maintained. The fibrinolytic system dissolves the fibrin clot with special enzymes (Figure 39-6).

 

 

The key event of fibrinolysis is the conversion of plasminogen to plasmin. Plasmin, an active enzyme, digests fibrin, fibrinogen, prothrombin, and factors V, VIII, and XII, thus breaking down the fibrin clot.

 

Hematologic Changes Associated with Aging 

 

Aging changes the cellular and plasma components of blood, making accurate assessment of the hematologic system in older adults more difficult. Chart 39-1 lists assessment tips for this population.

 

 

Several factors cause a decreased blood volume in older people. Total body water is decreased among older adults. In addition, they tend to have a lower concentration of plasma proteins and a decreased plasma osmotic pressure (possibly related to a decreased dietary intake of proteins), which also causes some loss of blood volume into the interstitial space. Bone marrow produces fewer blood cells as it ages. Total red blood cell (RBC) and white blood cell (WBC) counts (especially lymphocyte counts) are lower among older adults. Platelet counts do not appear to change with age. Lymphocytes become less reactive to antigens and have a loss of im mune function. Antibody levels and responses are lower in older adults. The leukocyte count does not rise as high in response to infection in older people as it does in young people (Workman, Ellerhorst-Ryan, & Koertge, 1993). Hemoglobin levels also change with age. Hemoglobin levels in men and women fall after middle age. Iron-deficient diets may play a role in this phenomenon.

 

Anticoagulants and Thrombolytics 

 

Drug therapy is commonly used to alter clotting ability or to destroy existing clots when circulation is at risk. The two broad categories of drugs are anticoagulants and thrombolytics. Both categories of agents are used widely to treat excessive or inappropriate clot formation, but their actions, side effects, and precautions are very different.

 

ANTICOAGULANTS

 

Anticoagulant drugs exert their effects by interfering with one or more steps in the blood-clotting cascade. Thus these agents prevent the formation of new clots and limit or prevent the extension of formed clots. Anticoagulants have no degradative effects on existing clots. Anticoagulants are further categorized into heparin, vitamin K antagonists, and platelet aggregation inhibitors. Table 39-3 summarizes the actions of different anticoagulants. Figure 39-4 shows where in the blood-clotting cascade these agents exert their anticlotting effects.

 

THROMBOLYTICS

 

Thrombolytic agents preferentially degrade fibrin threads already present in the formed blood clot. The four most extensively used agents are tissue plasminogen activator (t-PA), streptokinase (SK), reteplase, and anistreplase. The mechanism to start fibrin degradation is the activation of the inactive tissue protein plasminogen to its active form, plasmin. Plasmin directly attacks and degrades the fibrin molecule and has fewer effects on the fibrinogen molecule. The general action of all these thrombolytic agents is the selective degradation of formed fibrin clots with minimal effect on clot formation.

In general, the administration of thrombolytic agents results in clot breakdown with less disruption of blood clotting. Thrombolytic agents are the first-line therapy for conditions caused by existing small or localized formed clots, such as myocardial infarction, limited arterial thrombosis, thrombotic strokes, and occluded shunts. For some conditions (e.g., myocardial infarction), these drugs are given only within the first 6 hours after the onset of symptoms. This time limitation is not related to drug activity, because thrombolytic agents can break down clots older than 6 hours. Rather, tissue that has been anoxic for more than 6 hours is not likely to benefit from this therapy, making the risks to the client greater than the advantages.

Thrombolytic therapy has a very limited role when clotting is extensive, such as in deep vein thrombophlebitis within the pelvis or massive pulmonary emboli. In these situations clots are very large compared to their usual size in the coronary arteries. The amount of drug and duration of therapy needed for the effective breakdown of such clots is both cost prohibitive and physiologically risky. Many of these clots are not easily accessed for direct infusion of the thrombolytic agent. In addition, these clots are large, and the danger of releasing largesized particles from the clots during breakdown and having them occlude other blood vessels is much greater than the thrombolytic therapy of smaller arterial clots.

 

 ASSESSMENT TECHNIQUES

 

History

DEMOGRAPHIC DATA Chart 39-2 lists questions based on Gordon’s Functional Health Patterns to ask during the assessment of hematologic function. Age and gender are important variables to obtain when assessing the client’s hematologic status. Bone marrow and immune activity diminish with ag It is also important for the nurse to collect information on the client’s occupation, hobbies, and location of housing. This information may indicate an exposure to agents or chemicals that affect bone marrow growth and hematologic function.

PERSONAL AND FAMILY HISTORY Obtaining an accurate family history is important because many bleeding disorders are inherited. The nurse asks whether anyone in the family has had hemophilia, frequent nosebleeds, postpartum hemorrhages, excessive bleeding after tooth extractions, or continuous heavy bruising in response to relatively mild trauma. Family information about sickle cell disease or sickle cell traits also is obtained. Although sickle cell disease is seen primarily among African Americans, anyone may have the trait. Personal factors to be included in the hematologic assessment are liver function, the presence of known immunologic or hematologic disorders, and current medication use. Because liver function is important in the synthesis of clotting factors, the nurse also asks about jaundice, anemia, and gallstones.

 The client is asked about use of blood “thinners” such as sodium warfarin (Coumadin, Warfilone), aspirin, and other nonsteroidal anti-inflammatory drugs (NSAIDs). A person who takes aspirin on a daily basis may have bleeding problems, and many over-the-counter medications contain aspirin or other salicylates that disrupt platelet aggregation. The nurse determines all medications that the client is using or has used in the past 3 weeks. Clients are also asked about the use of antibiotics, because prolonged antibiotic therapy can lead to coagulopathies or bone marrow depression. Table 39-4 lists drugs known to alter hematologic function. Previous radiation therapy may result in some permanent impairment of hematologic function, especially if marrow-forming bones were in the path of the radiation.

DIET HISTORY Dietary pattern can alter cell quality and affect blood clotting. The nurse asks clients to record everything eaten during the previous week. This information is helpful in determining the causes of anemias and of protein, mineral, or vitamin deficiencies. Diets high in fat and carbohydrates and low in protein, iron, and vitamins can cause many types of anemia and decrease the functions of all blood cells. Clients are asked about alcohol consumption. Chronic alcoholism is associated with nutritional deficiencies and liver impairment, both of which can decrease the ability of the blood to clot. Certain dietary habits can enhance blood clotting. Diets high in vitamin K may increase the rate of blood coagulation. The nurse assesses the amount of raw, leafy green vegetables that the client consumes and whether he or she routinely takes supplemental vitamins. The amount of calcium consumed within the diet or in supplements is also assessed.

 

SOCIOECONOMIC STATUS

 

The nurse assesses the client’s ability to understand and follow instructions related to proper diet, specific procedures and tests, and therapeutic regimens. Personal resources, such as finances and social support, are determined. A person with a marginal income may have a diet low in iron and protein. The nurse also notes the client’s occupation and asks about potential exposure to chemicals.

 

CURRENT HEALTH PROBLEMS

 

The nurse determines whether the client has experienced swelling of the lymph nodes or excessive bruising or bleeding and whether the bleeding was spontaneous or induced by trauma. The client is asked about the amount and duration of bleeding after routine dental work. Women are asked about the presence of menorrhagia (excessive menstrual flow). They are asked to estimate the number of pads or tampons used during the most recent menstrual cycle and whether this amount represents a change from their usual pattern of menstrual flow. The nurse asks whether clots are present in menstrual blood. Clot size is estimated using coins or fruit for comparison (“clots are dime sized” or “clots are the size of lemons”). The nurse determines whether the client experiences dyspnea on exertion, palpitations, frequent infections, fevers, recent weight loss, headaches, or paresthesias. Any or all of these symptoms may accompany hematologic disease. The single most common symptom of anemia is fatigue. Clients are asked about feeling tired, needing more rest, or losing endurance during normal activities. They are asked to compare the extent and intensity of their activities during the past month with those of the same month a year ago. The nurse asks about other symptoms associated with anemia, such as vertigo, tinnitus, anorexia, dysphagia, and a sore tongue.

 

Physical Assessment 

 

The nurse performs a comprehensive physical assessment, because hematologic dysfunction affects the whole body. Certain problems are specific for hematologic assessment in older clients (see Chart 39-1).

 

SKIN ASSESSMENT

 

The nurse inspects the color of the skin for pallor or jaundice and of the mucous membranes and nail beds for pallor or cyanosis. Pallor of the gums, conjunctivae, and palmar creases indicates decreased hemoglobin levels. The gums are also assessed for active bleeding in response to light pressure or brushing the teeth with a soft-bristled brush, and any lesions or draining areas are noted. The nurse assesses for signs of bleeding in the form of petechiae and large bruises (ecchymoses). Petechiae are pinpoint hemorrhagic lesions in the skin. Bruises may be confluent or clustered. For hospitalized clients, the nurse determines if there is bleeding from sites such as nasogastric tubes, endotracheal tubes, central lines, peripheral intravenous sites, or Foley catheters. Skin turgor and itching are noted, because dry skin or intense itching can indicate hematologic disease.

 

HEAD AND NECK ASSESSMENT

 

The nurse notes pallor or ulceration of the oral mucosa. The tongue may be completely smooth in pernicious anemia and iron deficiency anemia or smooth and red iutritional deficiencies. These manifestations may be accompanied by fissures at the corners of the mouth. The nurse also observes for jaundice of the sclera. All lymph node areas are inspected and palpated. Any lymph node enlargement is documented, including whether palpation of the enlarged node causes pain. It is important to determine whether the enlarged node moves or remains fixed with palpation. 

 

RESPIRATORY ASSESSMENT

 

The rate and depth of respiration are assessed while the client is at rest, as well as during and after mild physical activity (e.g., walking 20 steps in 10 seconds). The nurse notes whether the client can complete a 10-word sentence without stopping for a breath. The nurse assesses whether the client is fatigued easily, experiences shortness of breath at rest or on exertion, or requires additional pillows to sleep comfortably at night. Many anemias cause these symptoms.

 

CARDIOVASCULAR ASSESSMENT

 

The nurse observes for heaves, distended neck veins, edema, or signs of phlebitis. He or she also auscultates for murmurs, gallops, irregular rhythms, and abnormal blood pressure. Systolic blood pressure tends to be lower thaormal in clients with anemia. In conditions of hypercellularity, blood pressure is greater thaormal. Severe anemias can cause right-sided ventricular hypertrophy and heart disease.

 

RENAL AND URINARY ASSESSMENT

 

The kidneys are extremely vascular, and bleeding problems may manifest as overt or occult hematuria (blood in the urine). The nurse inspects a voided sample of urine for color. Hematuria may be detected by grossly, bloody red or dark, brownish gold urine. The urine is tested for proteins with a urine test dipstick because hematologic problems may increase the protein content of urine. The urine sample also is tested for occult blood (Hemoccult test).

 

MUSCULOSKELETAL ASSESSMENT

 

Rib or sternal tenderness is an important sign of hematologic malignancy. The nurse examines the superficial surfaces of all bones, including the ribs and sternum, by applying intermittent firm pressure with the fingertips. The client’s range of joint motion is assessed, and any swelling or joint pain is documented.

 

ABDOMINAL ASSESSMENT

 

The normal adult spleen is usually not palpable. An enlarged spleen is associated with many hematologic problems. An enlarged spleen may be detected by percussion, but palpation is more reliable. The spleen lies just beneath the abdominal wall and is identified by its movement during respiration. During palpation, the client lies in a relaxed, supine position while the nurse stands on the client’s right side and palpates the left upper quadrant. The nurse palpates gently and cautiously, because an enlarged spleen may be tender and easily ruptured. Palpating the edge of the liver in the right upper quadrant of the abdomen can detect hepatic enlargement, which is often associated with hematologic problems. A normal liver may be palpable as much as 4 to 5 cm below the right costal margin but is usually not palpable in the epigastrium. A common cause of anemia among older adults is a chronically bleeding gastrointestinal lesion. If the lesion or open area is located in the stomach or small intestine, obvious blood may not be visible in the stool, or such a small amount is passed each day that the client is not aware of it. Therefore a stool specimen is obtained for occult blood testing.

 

CENTRAL NERVOUS SYSTEM ASSESSMENT

 

A thorough examination of the cranial nerves and neurologic function is necessary in many clients with hematologic disease. Vitamin B12 deficiency impairs cerebral, olfactory, spinal cord, and peripheral nerve function, and severe chronic deficiency may lead to irreversible neurologic degeneration. Many neurologic problems may develop in clients who have hematologic malignancies as a consequence of bleeding, infection, or tumor spread. When the client has a known or suspected bleeding disorder and has experienced any head trauma, the nurse expands the physical assessment to include frequent neurologic checks and mental status examinations. Other important clinical manifestations associated with impaired hematologic function include fever, chills, and night sweats.

 

Psychosocial Assessment 

 

The person with hematologic abnormalities may have a chronic illness (e.g., hemophilia or cancer) or an acute exacerbation of a chronic disease (e.g., pernicious anemia). In either instance each person brings his or her own coping style to the illness. After developing a rapport with the client, the nurse can learn what coping mechanisms he or she has used successfully during past illness or crises. The nurse also asks the client and family members about social support networks, community resources, and financial health. A problem in any of these areas can interfere with compliance with therapy and, ultimately, recovery.

 

Diagnostic Assessment

 

LABORATORY TESTS

 

In hematologic disease, the most definitive signs are often the laboratory test results. Chart 39-3 lists laboratory data associated with hematologic function.

 

Tests of Cell Number and Function

 

 COMPLETE BLOOD COUNT 

 

A complete blood count (CBC) includes a number of studies: red blood cell (RBC) count, white blood cell (WBC) count, hematocrit, and hemoglobin level. The RBC count measures circulating RBCs in 1 mm3 of venous blood, and the WBC count measures all leukocytes present in 1 mm3 of venous blood. To determine the percentages of different types of leukocytes circulating in the blood, a WBC count with differential leukocyte count is performed (see Chapter 20). The hematocrit (Hct) is calculated as the percentage of RBCs in the total blood volume, and the hemoglobin level represents the total amount of hemoglobin in the peripheral blood. The CBC can measure other variables of the circulating cells, including mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), and mean corpuscular hemoglobin concentration (MCHC). MCV measures the average volume or size of a single RBC and is useful for classifying anemias. When MCV is elevated, the cell is said to be abnormally large (macrocytic), as seen in megaloblastic anemias. When MCV is decreased, the cell is abnormally small(microcytic), as seen in iron deficiency anemia. MCH is the average amount of hemoglobin in a single RBC. MCHC measures the average concentration of hemoglobin in a single RBC. When the MCHC is decreased, the cell has a hemoglobin deficiency and is hypochromic, as in iron deficiency anemia.

 

RETICULOCYTE COUNT 

 

Another hematologic test helpful in determining bone marrow function is the reticulocyte count. A reticulocyte is an immature RBC. An elevated reticulocyte count indicates increased RBC production by the bone marrow. Normally about 2% of circulating RBCs are reticulocytes. An elevated reticulocyte count is desirable in an anemic client or after hemorrhage, because this indicates that the bone marrow is responding appropriately to a decrease in the total RBC mass. An elevated reticulocyte count without a precipitating cause may indicate a pathologic condition, such as polycythemia vera.

 

 HEMOGLOBIN ELECTROPHORESIS 

 

Hemoglobin electrophoresis detects abnormal forms of hemoglobin, such as hemoglobin S in sickle cell disease. Hemoglobin A is the major component of hemoglobin in the normal RBC. Decreased levels of hemoglobin A with increasing levels of other types of hemoglobin are typical of some hematologic problems, such as sickle cell disease.

 

LEUKOCYTE ALKALINE PHOSPHATASE 

 

Leukocyte alkaline phosphatase (LAP) is an enzyme produced by normal mature neutrophils. Elevated LAP levels occur during episodes of infection or stress. An elevated neutrophil count without an accompanying elevation in LAP level is associated with chronic myelogenous leukemia.

 

COOMBS’ TEST 

 

The two Coombs’ tests (direct and indirect) are used for blood typing. The direct test detects the presence of antibodies (also called antiglobulins) against RBCs that may be attached to the RBCs. Although healthy people can make these antibodies, certain diseases (e.g., systemic lupus erythematosus, mononucleosis, and lymphomas) are associated with the production of antibodies directed against the body’s own RBCs. The presence of these antibodies usually causes a hemolytic anemia. The indirect Coombs’ test detects the presence of circulating antiglobulins. The test is used to determine whether the client has serum antibodies to the type of RBCs he or she is about to receive by blood transfusion.

 

SERUM FERRITIN, TRANSFERRIN, AND TOTAL IRON-BINDING CAPACITY 

 

Serum ferritin, transferrin, and total iron-binding capacity (TIBC) tests measure iron levels. Abnormal levels of iron and TIBC are associated with many hematologic problems, including iron deficiency anemia. The serum ferritin test measures the quantity of iron present as free iron in the plasma. The amount of serum ferritin is proportionally related to the amount of intracellular iron and represents 1% of the total body iron stores. Therefore the serum ferritin level provides a means to assess total iron stores. People with serum ferritin levels within 10 g of the normal range for their gender have adequate iron stores; people with levels 10 g or more lower than the normal range have inadequate iron stores and have difficulty recovering from any hemorrhagic event. Transferrin is a protein that transports iron from the gastrointestinal tract to cell storage sites. It is not easy to measure the amount of transferrin. However, measuring the amount of iron that can be bound to serum transferrin indirectly determines whether an adequate amount of transferrin is present. This test is the TIBC test. In healthy people, only about 30% of the transferrin is bound to iron in the blood. TIBC is measured by taking a sample of blood and adding measured amounts of iron to it. TIBC is calculated when the blood no longer binds the iron but allows it to precipitate. TIBC increases when a person is deficient in serum iron and stored iron levels. Such a value indicates that an adequate amount of transferrin is present but that less than 30% of it is bound to serum iron. I Tests Measuring Bleeding and Coagulation

 

CAPILLARY FRAGILITY TEST 

 

The capillary fragility test, or Rumpel-Leede test, measures vascular hemostatic function by increasing intracapillary pressure in the arm. This is done by occluding venous outflow or by applying controlled negative pressure to a skin area. A blood pressure cuff is usually inflated to a pressure halfway between the systolic and diastolic pressures. This pressure is maintained for 5 minutes, and the petechiae that appear distal to the cuff are counted. Normally, five to ten petechiae appear. The capillary fragility test can help determine whether excessive bleeding or bruising results from increased capillary fragility or impaired platelet action.

 

 BLEEDING TIME TEST 

 

The bleeding time test evaluates vascular and platelet activity during hemostasis. A special spring-loaded lancet that ensures uniform wound depth is used to make a small incision in the forearm while a blood pressure cuff remains inflated at 40 mm Hg. Blood is blotted from the site at 30-second intervals, and the time required for the bleeding to stop is recorded. Normal bleeding time ranges from 1 to 9 minutes.

 

PROTHROMBIN TIME 

 

Prothrombin time (PT) is a measurement of how long blood takes to clot. This test reflects how much of the clotting factors II, V, VII, and X is present and how well they are functioning. When sufficient amounts of these clotting factors are present and functioning, the PT shows blood clotting between 11 and 13 seconds, or within 85% to 100% of the time needed for a control sample of blood to clot. PT is prolonged when one or more clotting factors is deficient, or when liver disease is present. Sodium warfarin (Coumadin, Warfilone’+O therapy is also monitored using PT levels. Appropriate warfarin therapy prolongs PT by 1.5 to 2 times the client’s normal PT value. Facilities are using the PT test less often to assess blood clotting because control blood is taken from different people and may not be the same from one day to the next, even in a single laboratory. To eliminate PT errors resulting from variations in control blood or in some of the chemicals used in the test, the International Normalized Ratio (INR) is used more often to assess clotting time.

 

INTERNATIONAL NORMALIZED RATIO 

 

The INR measures the same process as the PT but in a slightly different way—by establishing a normal mean or standard for PT. The INR is calculated by dividing the client’s PT by the established standard PT. A normal INR ranges between 0.7 and 1.8. When using the INR to monitor warfarin therapy, it should be maintained between 2.0 and 3.0 regardless of the actual PT in seconds.

 

PARTIAL THROMBOPLASTIN TIME 

 

Partial thromboplastin time (PTT) assesses the intrinsic coagulation cascade and evaluates the presence of factors II, V, VIII, IX, XI, and XII. PTT is prolonged whenever any of these factors is deficient, such as in hemophilia or disseminated intravascular coagulation (DIC). Because factors II, IX, and X are vitamin K dependent and are produced in the liver, liver disease can decrease their concentration and prolong PTT. Heparin (Calciparine, Liquaemin, Hepalean^) therapy is monitored by PTT. The desired ranges for therapeutic anticoagulation are 1.5 to 2.5 times the normal values. Controversy exists regarding whether this test is accurate when the blood sample is obtained through an existing vascular access device (e.g., arterial line or normal saline lock) instead of through a separate new venipuncture. Clinical studies have shown that with an appropriate discard, samples obtained through existing vascular access devices do accurately reflect the client’s activated partial thromboplastin time (see the Evidence-Based Practice for Nursing box at right).

 

PLATELET AGGLUTINATION/AGGREGATION 

 

Platelet aggregation, or the ability to clump, can be tested by mixing the client’s plasma with a substance called ristocetin. The degree of aggregation is noted. Aggregation can be impaired in von Willebrand’s disease and during the use of drugs such as aspirin, anti-inflammatory agents, and psychotropic agents. 1

 

RADIOGRAPHIC EXAMINATIONS

 

Assessment of the client with a suspected hematologic abnormality can include radioisotopic imaging. Isotopes are used to evaluate the bone marrow for sites of active blood cell formation and sites of iron storage. Radioactive colloids are routinely used to determine organ size and liver and spleen function. The client is given a radioactive isotope intravenously approximately 3 hours before the procedure. The client is taken to the nuclear medicine department for the scan, where he or she must lie still for approximately 1 hour. No special client preparation or follow-up care is needed for these tests. Standard x-ray studies may be used to diagnose certain hematologic disorders. For example, multiple myeloma causes characteristic bone destruction, with a “Swiss cheese” appearance on the x-ray film.

 

BONE MARROW ASPIRATION AND BIOPSY

 

A bone marrow aspiration or biopsy is often performed to evaluate the client’s hematologic status when other tests show persistent abnormal findings. Results can provide important information about bone marrow function, including the production of red blood cells (RBCs), white blood cells (WBCs), and platelets. Bone marrow aspiration and bone marrow biopsy are similar invasive procedures. In a bone marrow aspiration, cells and fluids are suctioned from the bone marrow. In a bone marrow biopsy, solid tissue and cells are obtained by coring out an area of bone marrow with a large-bore needle. A physician’s order and a signed, informed consent are obtained from the client before a bone marrow aspiration or biopsy is performed. Bone marrow aspiration may be performed by a physician, sanctioned clinical nurse specialist, nurse practitioner, or physician assistant, depending on the agency’s policy and regional law. The procedure may be performed at the client’s bedside, in an examination room, in a laboratory, or in a clinic setting. On learning what specific tests will be performed on the marrow, the nurse checks the facility’s procedure manual and checks with the hematology laboratory to determine how to handle the specimen. Some tests require the addition of heparin or other special solutions to the specimen.

 

CLIENT PREPARATION. 

 

Most clients experience anxiety or fear before a bone marrow aspiration. Clients who have experienced a bone marrow aspiration may have less anxiety or more anxiety depending on their previous experience. The nurse can help reduce anxiety and allay fears by providing accurate information and continuous emotional support. Some clients like to have their hand held during the procedure; other clients may want the nurse to hug or hold their entire upper body. The nurse explains the procedure to the client and says that he or she will stay during the entire procedure. Occasionally, a friend or family member is permitted to be pres ent to hold the client’s hand and provide additional emotional support. If a local anesthetic is used, the nurse explains that the injection will feel like a stinging or burning sensation. The nurse tells the client to expect a heavy sensation of pressure and pushing while the needle is being inserted. Some clients also can hear a crunching sound or feel a scraping sensation as the needle punctures the bone. The nurse explains that a brief sensation of painful pulling will be experienced as the marrow is aspirated by mild suction in the syringe. If a biopsy is performed, the client may feel more pressure and discomfort as the needle is rotated into the bone. The client is assisted onto an examining table, and the site (most commonly the iliac crest) is exposed. If this site is not available or if more marrow is needed, the sternum can be used. If the iliac crest is being used, the client is usually placed in the prone position or occasionally in the side-lying position. Depending on the tests to be performed on the specimen, a laboratory technician may also be present to ensure appropriate handling of the specimen.

 

PROCEDURE. 

 

The procedure usually lasts from 5 to 15 minutes. Clients may be uncomfortable and may experience pain. The type and amount of anesthesia or sedation used depends on the physician’s preference, the client’s preference and previous experience with bone marrow aspiration and biopsy, and the setting. A local anesthetic solution may be injected into the skin around the site. The client may also receive a mild tranquilizer or a rapid-acting sedative, such as midazolam hydrochloride (Versed) or lorazepam (Ativan, Apo-Lorazepam+, Novo-Lorazem). Some clients do well with guided imagery or autohypnosis. Aspiration or biopsy procedures are invasive, and sterile precautions are observed. The skin over the site is cleaned with a disinfectant solution. For an aspiration, the needle is inserted with a twisting motion, and the marrow is aspirated by pulling back on the plunger of the syringe. When sufficient marrow has been aspirated to ensure accurate analysis, the needle is carefully and rapidly withdrawn while the tissues are supported at the site. For a biopsy, a small skin incision is made, and the biopsy needle is inserted through the skin opening. Pressure and several twisting motions are performed to ensure coring and loosening of an adequate amount of marrow tissue. External pressure is applied to the site until hemostasis is ensured. A pressure dressing or sandbags may be applied to minimize bleeding at the site.

 

FOLLOW-UP CARE. 

 

The site is covered with a dressing after hemostasis is achieved and is observed closely for 24 hours for signs of bleeding and infection. A mild analgesic (aspirin-free) is prescribed for discomfort, and ice packs are applied over the site to limit bruising. The nurse instructs the client to inspect the site every 2 hours for the first 24 hours and to note the presence of active bleeding or bruising. The client is advised to avoid contact sports or any activity that might result in trauma to the site for 48 hours. Information obtained from bone marrow aspiration or biopsy reflects the degree and quality of bone marrow activity. The counts made on a marrow specimen can indicate whether stem cells, blast cells, committed cells, and more mature cell forms are present in the expected quantities and proportions. In addition, bone marrow aspiration or biopsy can confirm the spread of cancer cells from other tumor sites.   

 

 

Disorders of the hematologic system can occur as a result of problems in the production, function, or normal destruction of any type of blood cell. The type and severity of the specific disorder determine the degree of threat to the client’s wellbeing. This chapter discusses hematologic conditions that may have only a minor impact on activities of daily living (ADLs), as well as those disorders that are potentially life threatening, such as sickle cell disease and leukemia.

 

Coagulation disorders are synonymous with bleeding disorders and are characterized by abnormal or increased bleeding resulting from defects in one or more components regulating hemostasis. Bleeding disorders may be spontaneous or traumatic, localized or generalized, lifelong or acquired. They can originate from a defect in the hemostatic processes at the vascular, platelet, or clotting factor level. Figure 40-4 outlines blood-clotting cascades and sites where specific defects and drugs disrupt the hemostatic processes.

 

PLATELET DISORDERS 

 

Platelets play a vital role in hemostasis. For both the intrinsic and the extrinsic pathways, blood clotting starts with platelet adhesion and the formation of a platelet plug. Any condition that either reduces the number of platelets or interferes with their ability to adhere (to one another, blood vessel walls, collagen, or fibrin threads) can be manifested as increased bleeding. Platelet disorders can be inherited, acquired, or temporarily induced by the ingestion of substances that limit platelet production or inhibit aggregation. A drop in the number of platelets below the level needed for normal coagulation is calledthrombocytopenia. Thrombocytopenia may occur as a result of other conditions or treatments that suppress general bone marrow activity. It also can occur through processes that specifically limit platelet formation or increase the rate of platelet destruction. The two thrombocytopenic conditions affecting adults are autoimmune thrombocytopenic purpura and thrombotic thrombocytopenic purpura.

 

Autoimmune Thrombocytopenic Purpura

 

OVERVIEW

 

Before the underlying cause of autoimmune thrombocytopenic purpura was identified, this condition was known as idiopathic thrombocytopenic purpura (ITP). Although the cause is now thought to be an autoimmune reaction, the condition is still commonly known as ITP. The total number of circulating platelets is greatly reduced in ITP, even though platelet production in the bone marrow is normal. Clients with idiopathic thrombocytopenic purpura make an antibody directed against the surface of their own platelets (an antiplatelet antibody). This antibody coats the surface of the platelets, making them more susceptible to attraction and destruction by phagocytic leukocytes, especially macrophages. Because the spleen contains a large concentration of macrophages and because the blood vessels of the spleen are long and twisted, antibody-coated platelets are destroyed primarily in the spleen. When the rate of platelet destruction exceeds that of production, the number of circulating platelets decreases and blood clotting slows. Although the cause of this disorder appears to be autoimmune, the exact mechanism initiating the production of autoantibodies is unknown. ITP is most common among women between the ages of 20 and 40 and among people with a preexisting autoimmune condition, such as systemic lupus erythematosus (Cotran, Kumar, & Robbins, 1999).

 

COLLABORATIVE MANAGEMENT

 

Assessment

 

Clinical manifestations associated with ITP are generally limited to the skin and mucous membranes: large ecchymoses(bruises) on the arms, legs, upper chest, and neck or a petechial rash. Mucosal bleeding occurs easily. If the client has experienced significant blood loss, signs of anemia may also be present. A rare complication is an intracranial bleeding-induced stroke. The nurse assesses for neurologic function and mental status. Family members or significant others are asked if the client’s behavior and responses to the mental status examination are typical or represent a change from usual reactions. Idiopathic thrombocytopenic purpura is diagnosed by a decreased platelet count and large numbers of megakaryocytes in the bone marrow. Antiplatelet antibodies may be present in detectable levels in peripheral blood. If the client experiences any episodes of bleeding, hematocrit and hemoglobin levels also are low.

Interventions

 

NONSURGICAL MANAGEMENT.

 

As a result of the decreased platelet count, the client is at great risk for bleeding. Interventions include therapy for the underlying condition, as well as protection from trauma-induced bleeding episodes.

 

DRUG THERAPY. 

 

Agents used to control ITP include drugs that suppress immune function to some degree. The premise for the use of agents such as corticosteroids and azathioprine (Imuran) is to inhibit immune system synthesis of antiplatelet autoantibodies. More aggressive therapy can include low doses of chemotherapeutic agents, such as the antimitotic agents and cyclophosphamide.

 

BLOOD REPLACEMENT THERAPY.

 

 For the client with a platelet count of less than 20,000/mm3 who is experiencing an acute life-threatening bleeding episode, a platelet transfusion may be required. Platelet transfusions are not performed routinely, because the donated platelets are just as rapidly destroyed by the spleen as the client’s own platelets (see later discussion under Platelet Transfusions, p. 865).

 

MAINTAINING A SAFE ENVIRONMENT.

 

 The nurse’s major objectives are to protect the client from situations that can lead to bleeding and to closely monitor the amount of bleeding that is occurring. (For nursing care actions, see Risk for Injury [Leukemia], p. 856.)

 

SURGICAL MANAGEMENT.

 

For the client who does not respond to drug therapy, splenectomy may be the treatment of choice. Because the leukocytes in the spleen perform many different immunodefensive functions, the client who has undergone a splenectomy is at increased risk for infection.

 

Thrombotic Thrombocytopenic Purpura

 

OVERVIEW

 

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder in which platelets clump together inappropriately in the microcirculation and insufficient platelets remain in the systemic circulation. The client experiences inappropriate clotting, yet the blood fails to clot properly when trauma occurs. The underlying cause of TTP appears to be an autoimmune reaction in blood vessel cells (endothelial cells) that makes platelets clump together in very small blood vessels. As a result, tissues become ischemic. Common manifestations include renal failure, myocardial infarction, and stroke. If left untreated, this condition is fatal within 3 months in 90% of clients (McBrien, 1997).

 

COLLABORATIVE MANAGEMENT

 

Treatment for the client with TTP focuses on inhibiting the inappropriate platelet aggregation and disrupting the underlying autoimmune process. Primary treatment consists of plasma pheresis with the infusion of fresh frozen plasma. This treatment provides the necessary platelet aggregate inhibitors (McBrien, 1997). Drugs that inhibit platelet clumping, such as aspirin, alprostadil (Prostin), and plicamycin, also may be helpful. Immunosuppressive therapy reduces the intensity of this disorder.

 

CLOTTING FACTOR DISORDERS

 

Coagulation or bleeding disorders can result from a clotting factor defect, including the inability to produce a specific clotting factor, production of insufficient quantities, or a less active form of clotting factor. Most clotting factor disorders are congenitally transmitted gene abnormalities of one clotting factor. The few acquired clotting factor disorders are related to an inability to synthesize many clotting factors at the same time as a result of liver damage or an insufficiency of clotting cofactors and precursor products. Common congenital disorders that result in defects at the clotting factor level include hemophilias A and B and von Willebrand’s disease. Disseminated intravascular coagulation (DIC) may be considered an acquired clotting disorder but is more closely associated with septic shock.

 

Hemophilia

 

OVERVIEW

 

Hemophilia comprises two hereditary bleeding disorders resulting from deficiencies of specific clotting factors. Hemophilia A (classic hemophilia) results from a deficiency of factor VIII and accounts for 80% of cases of hemophilia. Hemophilia B (Christmas disease) is a deficiency of factor IX and accounts for 20% of cases. The incidence of both is 1 in 10,000. Hemophilia is an Xlinked recessive trait. Female carriers have a 50% chance of transmitting the gene for hemophilia to their daughters (who then are carriers) and to their sons (who will have overt hemophilia). Hemophilia A is, with rare exceptions, a disease affecting males, none of whose sons will have the gene for hemophilia and all of whose daughters will be carriers (able to pass on the gene without actually having the disorder). In about 30% of clients with hemophilia, there is no family history, and it is presumed that their disease is the result of a new mutation (Cotran, Kumar, & Robbins, 1999). The bleeding disorder associated with hemophilia A is so severe that before blood transfusions were available, children with hemophilia rarely survived past age 3 years. With the availability of blood transfusion and factor VIII therapy, mean survival time has increased so greatly that hemophilia now is commonly seen among adult clients. The clinical pictures of hemophilia A and B are identical. The client has abnormal bleeding in response to any trauma because of an absence or deficiency of the specific clotting factor. Clients with hemophilia do not bleed more frequently or even more rapidly that those without the disease, but they do bleed for a longer period (Ligda, 1998). Hemophiliacs form platelet plugs at the bleeding site, but the clotting factor deficiency impairs the hemostatic response and the capacity to form a stable fibrin clot. This produces abnormal bleeding, which may be mild, moderate, or severe, depending on the degree of factor deficiency.

 

COLLABORATIVE MANAGEMENT

 

 Assessment

 

Assessment of the client with hemophilia reveals the following:

• Excessive hemorrhage from minor cuts or abrasions caused by abnormal platelet function

• Joint and muscle hemorrhages that lead to disabling long-term sequelae

• A tendency to bruise easily

• Prolonged and potentially fatal postoperative hemorrhage

The laboratory test results for a client with hemophilia demonstrate a prolonged partial thromboplastin time (PTT), a normal bleeding time, and a normal prothrombin time (PT) (Cotran, Kumar, & Robbins, 1999). The most common health problem associated with hemophilia is degenerating joint function resulting from chronic bleeding into the joints, especially at the hip and knee.

 

Interventions 

 

The bleeding problems of hemophilia A can be well managed by either regularly scheduled IV administration of factor VIII cryoprecipitate or intermittent administration as needed, depending on the individual’s activity level and injury probability (see Cryoprecipitate, p. 865). However, the cost of cryoprecipitate is prohibitive for many people with hemophilia. In addition, because the precipitated clotting factors are derived from pooled human serum, a risk of viral contamination remains, even with the use of heat-inactivated serum (Ligda, 1998). Major complications of hemophilia therapy during the 1980s were infection with hepatitis B virus, cytomegalovirus, and human immunodeficiency virus (HIV). Although heat-inactivated serum and the elimination of HIV-positive donors have reduced these risks, they have not yet been eliminated.

 

TRANSFUSION THERAPY

 

Any blood component may be removed from a donor and transfused to benefit a recipient. Components may be transfused individually or collectively, with varying degrees of benefit to the recipient.

 

PRETRANSFUSION RESPONSIBILITIES 

 

Nursing actions during transfusions aim largely at prevention or early recognition of adverse transfusion reactions. Preparation of the client for transfusion therapy is imperative, and in stitutional blood product administration procedures should be carefully followed. Before administering any blood product, the nurse reviews the agency’s policies and procedures. Chart 40-15 presents best practices for transfusion therapy.

 

 Legally, a physician’s order is needed to administer blood or its components. The order specifies the type of component to be delivered, the volume to be transfused, and any special conditions the physician judges to be important. The nurse verifies the order for accuracy and completeness. The nurse also evaluates the need for transfusion, considering both the client’s clinical condition and the laboratory values. In many hospitals a separate consent form must be obtained for the administration of blood products before a transfusion is performed.

A blood specimen is obtained for crossmatching (testing of the donor’s blood and the recipient’s blood for compatibility). The procedure and responsibility for obtaining this specimen are specified by hospital policy. The laboratory requires at least 45 minutes to complete the crossmatch testing. In most hospitals a new crossmatching specimen is required at least every 48 hours (Dreger & Tremback, 1998).

Because of the viscosity of blood components, a 19-gauge needle or larger is used, whenever possible, for venous access. Both Y-tubing and straight tubing sets are available for blood component administration. A blood filter (approximately 170 xm) to remove aggregates from the stored blood products is included with component administration equipment and must be used to transfuse all blood products. In massive transfusion, a microaggregate filter (20 to 40 jam) may be used.

Normal saline is the solution of choice for administration. Ringer’s lactate and dextrose in water are contraindicated for administration with blood or blood products because they cause clotting or hemolysis of blood cells.

 

 Medications are never added to blood products.

 Before the transfusion is initiated, it is essential to determine that the blood component delivered is correct. Two registered nurses simultaneously check the physician’s order, the client’s identity, and whether the hospital identification band name and number are identical to those on the blood component tag. The blood bag label, the attached tag, and the requisition slip are examined to ensure that the ABO and Rh types are compatible. The expiration date is also checked, and the product is inspected for discoloration, gas bubbles, or cloudiness—indicators of bacterial growth or hemolysis.

 

TRANSFUSION RESPONSIBILITIES 

 

The nurse takes the vital signs, including temperature, immediately before initiating the transfusion. Infusion begins slowly. A nurse remains with the client for the first 15 to 30 minutes. Any severe reaction usually occurs with administration of the first 50 mL of blood. The nurse assesses vital signs 15 minutes after initiation of the transfusion to detect signs of a reaction. If there are none, the infusion rate can be increased to transfuse 1 unit in about 2 hours (depending on the client’s cardiovascular status). The nurse takes the vital signs every hour throughout the transfusion or as specified by agency policy. Blood components without large amounts of red blood cells (RBCs) can be infused more quickly. The identification checks are the same as for RBC transfusions. Physiologic changes in older clients may necessitate that blood products be transfused at a slower rate. Best practices related to the nursing care needs of older clients undergoing transfusion therapy are provided in Chart 40-16.

 

TYPES OF TRANSFUSIONS

 

Red Blood Cell Transfusions 

 

RBCs are administered to replace erythrocytes lost as a result of trauma or surgical interventions. Clients with clinical conditions that result in the destruction or abnormal maturation of RBCs may also benefit from RBC transfusions. Packed RBCs, supplied in 250-mL bags, are a concentrated source of RBCs and are the most common component administered to RBCdeficient clients (Dreger & Tremback, 1998). Packed RBCs are administered to individuals with a hemoglobin concentration less than 6 g/dL (or a hemoglobin value of 6 to 10 g/dL if clinical symptoms are present) (Kennedy, 1999). Blood transfusions are actually transplantations of tissue from one person to another. The donor and recipient blood must thus be carefully checked for compatibility to prevent potentially lethal reactions (Table 40-7).

 

Compatibility is determined by two different types of antigen systems (cell surface proteins): the ABO system antigens and the Rh antigen, present on the membrane surface of RBCs (Dreger & Tremback, 1998). RBC antigens are inherited.

For the ABO antigen system, a person inherits one of the following:

• A antigen (type A blood)

• B antigen (type B blood)

• Both A and B antigens (type AB blood)

• No antigens (type O blood)

Within the first few years of a child’s life, circulating antibodies develop against the blood type antigens that were not inherited. For example, a child with type A blood will form antigens against type B blood. A child with type O blood has not inherited either A or B antigens and will form antibodies against RBCs that contain either A or B antigens. If erythrocytes that contain a foreign antigen are infused into a recipient, the donated tissue can be recognized by the immune system of the recipient as non-self, and the client may have a reaction to the transfused products. The mechanism of the Rh antigen system is slightly different. An Rh-negative person is born without the antigen and does not form antibodies unless he or she is specifically sensitized to it. Sensitization can occur with RBC transfusions from an Rh-positive person or from exposure during pregnancy and birth. Once an Rh-negative person has been sensitized and antibody development has occurred, any exposure to Rh-positive blood can cause a transfusion reaction. Antibody development can be prevented by administration of Rh-immune globulin as soon as exposure to the Rh antigen is suspected. People who have Rh-positive blood can receive an RBC transfusion from an Rh-negative donor, but Rh-negative people must never receive Rh-positive blood.

 

Platelet Transfusions 

 

Platelets are administered to clients with platelet counts below 20,000 mm3 and to clients with thrombocytopenia who are actively bleeding or are scheduled for an invasive procedure (Dreger, & Tremback, 1998). Platelet transfusions are usually pooled from as many as 10 donors and do not have to be of the same blood type as the client. For clients who are candidates for bone marrow transplantation (BMT) or who require multiple platelet transfusions, single-donor platelets may be ordered. Single-donor platelets are obtained from one person and decrease the amount of antigen exposure to the recipient, helping prevent the formation of platelet antibodies. The chances of allergic transfusion reactions to future platelet transfusions are thus reduced. Platelet infusion bags usually contain 300 mL for pooled platelets and 200 mL for single-donor platelets. Because the platelet is a fragile cell, platelet transfusions are administered rapidly after being brought to the client’s room, usually over a 15- to 30-minute period. A special transfusion set with a smaller filter and shorter tubing is used. Standard transfusion sets are not used with platelets because the filter traps the platelets, and the longer tubing increases platelet adherence to the lumen. Additional platelet filters help remove white blood cells (WBCs) in the platelet concentrate. These filters are connected directly to the platelet transfusion set and are used for clients who have a history of febrile reactions or who will require multiple platelet transfusions. The nurse takes the vital signs before the infusion, 15 minutes after the infusion is initiated, and at its completion. The client may be premedicated with meperidine (Demerol) or hydrocortisone to minimize the chances of a reaction. He or she can become febrile and experience rigors(severe chills) during transfusion, but these symptoms are not considered a true transfusion reaction. IV administration of amphotericin B (Amphotec, Fungizone), an antifungal agent given to many clients with leukemia, is discontinued during platelet transfusion and not resumed for at least 1 hour after transfusion. Amphotericin B can cause severe allergic reactions that are difficult to distinguish from transfusion reactions.

 

 Plasma Transfusions

 

 Historically, plasma infusions have been administered to replace blood volume, and they are occasionally still used for this purpose. It is more common for plasma to be frozen immediately after donation. Freezing preserves the clotting factors, and the plasma can then be used for clients with clotting disorders (Kennedy, 1999).

Fresh frozen plasma (FFP) is infused immediately after thawing while the clotting factors are still viable. Clients who are actively bleeding with a prothrombin time (PT) or partial thromboplastin time (FIT) greater than 1.5 times normal are candidates for an FFP infusion (Harrahill & DeLoughery, 1998). ABO compatibility is required for transfusion of plasma products. The volume of the infusion bag is approximately 200 mL. The infusion takes place as rapidly as the client can tolerate, generally over a 30- to 60-minute period, through a regular Y-set or straight-filtered tubing.

 

Cryoprecipitate is a product derived from plasma. Clotting factors VIII and XIII, von Willebrand’s factor, fibronectin, and fibrinogen are precipitated from pooled plasma to produce cryoprecipitate. Clients with a fibrinogen level less than 100 mg/dL are candidates for a cryoprecipitate infusion (Harrahill & DeLoughery, 1998). This highly concentrated blood product is administered to clients with clotting factor disorders at a volume of 10 to 15 mL/unit. Although cryoprecipitate can be infused, it is usually given by IV push within 3 minutes. Dosages are individualized, and it is best if the cryoprecipitate is ABO compatible.

 

Granulocyte Transfusions 

At some centers, neutropenic clients with infections receive granulocyte transfusions for WBC replacement. However, this practice is highly controversial because the potential benefit to the client must be weighed against the potential severe reactions that often accompany granulocyte transfusions. The surfaces of granulocytes contaiumerous antigens that can cause severe antibody-antigen reactions when infused into a recipient whose immune system recognizes these antigens as non-self. In addition, transfused granulocytes have a very short life span and are probably of minimal benefit to the client (see Chapter 20). There is some evidence that treatment with antibiotics alone results in better survival rates. Granulocytes are suspended in 400 mL of plasma and should be transfused over a 45- to 60- minute period. Institutional policies often require more stringent monitoring of clients receiving granulocytes. A physician may need to be present in the hospital unit, and vital signs may need to be taken every 15 minutes throughout the transfusion. Administration of amphotericin B and granulocyte transfusions should be separated by 4 to 6 hours.

 

TRANSFUSION REACTIONS 

Clients can experience any of the following transfusion reactions: hemolytic, allergic, febrile, or bacterial reactions; circulatory overload; or transfusion-associated graft-versus-host disease (GVHD). The nurse is vigilant to prevent serious complications through early detection and initiation of appropriate treatment.

 

Hemolytic Transfusion Reactions 

Hemolytic transfusion reactions are caused by blood type or Rh incompatibility. When blood containing antibodies against the recipient’s blood is infused, antigen-antibody complexes are formed and released into the circulation. These complexes can destroy the transfused cells and initiate inflammatory responses in the recipient’s blood vessel walls and organs. The ensuing reaction may be mild, with fever and chills, or life threatening, with disseminated intravascular coagulation (DIC) and circulatory collapse (Robb, 1999).

Other clinical signs include the following:

• Apprehension

• Headache

• Chest pain

• Low back pain

• Tachycardia

• Tachypnea.

• Hypotension

• Hemoglobinuria

• A sense of impending doom

The onset of a hemolytic transfusion reaction may be immediate or may not occur until subsequent units have been transfused.

 

Allergic Transfusion Reactions 

Allergic transfusion reactions are most often seen in clients with a history of allergy. They may have urticaria, itching, bronchospasm, or occasionally anaphylaxis. Onset of this type of reaction usually occurs during or up to 24 hours after the transfusion. Clients with a history of allergy can be given buffy coat-poor or washed red blood cells (RBCs) in which the white blood cells (WBCs) and plasma have been removed. This procedure minimizes the possibility of an allergic reaction.

 

Febrile Transfusion Reactions 

Febrile transfusion reactions occur most commonly in the client with anti-WBC antibodies, a situation seen after multiple transfusions. The recipient experiences the following:

• Sensations of cold

• Tachycardia

• Fever

• Hypotension

• Tachypnea

 

Again, the physician can order buffy coat-poor RBCs or single-donor HLA-matched platelets. Leukocyte filters may also be used to trap WBCs and prevent their transfusion into the client.

 

 Bacterial Transfusion Reactions 

Bacterial transfusion reactions are seen after transfusion of contaminated blood products. Usually a gram-negative organism is the source because these bacteria grow rapidly in blood stored under refrigeration. Symptoms include the following:

• Tachycardia

• Hypotension

• Fever

• Chills

• Shock

The onset of a bacterial transfusion reaction is rapid. 

 

Circulatory Overload 

Circulatory overload can occur when a blood product is administered too quickly (Goldy, 1998). This complication is most common with whole-blood transfusions or when the client requires multiple transfusions. Older adults are most at risk for this condition (see Chart 40-16). Symptoms include the following:

• Hypertension

• Bounding pulse

• Distended jugular veins

• Dyspnea

• Restlessness

• Confusion

The nurse can both manage and prevent this complication by monitoring intake and output, transfusing blood products more slowly, and administering diuretics.

 

Transfusion-Associated Graft-Versus-Host Disease 

Transfusion-associated graft-versus-host disease (TA-GVHD) is an infrequent but life-threatening complication that can occur in both immunosuppressed and immunocompetent clients. Its cause in immunosuppressed clients is similar to that of GVHD associated with allogeneic bone marrow transplantation (BMT), discussed on p. 855, in which donor T-cell lymphocytes attack host tissues. The cause of TA-GVHD in immunocompetent hosts is uncertain. Reactions are more common when the host and donor share similar human leukocyte antigens (HLAs), such as in first-degree relatives or individuals with a similar ethnic background. Symptoms typically occur within 1 to 2 weeks and include thrombocytopenia, anorexia, nausea, vomiting, chronic hepatitis, weight loss, and recurrent infection. TA-GVHD has a 90% mortality rate but can be prevented by using irradiated blood products, which destroy T-cells and their cytokine products (Dreger & Tremback, 1998).

 

AUTOLOGOUS BLOOD TRANSFUSIONS 

Autologous blood transfusions involve collection and transfusion of the client’s own blood. Advantages of this type of transfusion are guaranteed compatibility and elimination of the risk of transmitting diseases such as hepatitis or HIV. The four types of autologous blood transfusions are preoperative autologous blood donation, acute normovolemic hemodilution, intraoperative autologous transfusion, and postoperative blood salvage. Preoperative autologous blood donation, the most common type of autologous blood transfusion, involves collection of whole blood from the client, division into components, and then storage for later use (such as after a scheduled surgical procedure).

As long as hematocrit and hemoglobin levels are within a safe range, the client can donate blood on a weekly basis until the prescribed amount of blood is obtained. Fresh packed RBCs may be stored for 42 days. For individuals with rare blood types, blood may be frozen for up to 10 years. Platelets and plasma may be collected via pheresis. Some cardiovascular problems and bacteremia are contraindications for autologous blood donation. Acute normovolemic hemodilution involves withdrawal of a client’s RBCs and volume replacement just before a surgical procedure. The goal is to decrease RBC loss during surgery. The blood is stored at room temperature for up to 6 hours and reinfused after surgery. This type of autologous transfusion is appropriate for healthy clients but is contraindicated for individuals who are anemic or who have poor renal function. Intraoperative autologous transfusion and postoperative blood salvage involve the recovery and reinfusion of a client’s own blood, collected either from an operative field or postoperatively from a wound.

Several commercial products are available that collect, filter, and drain the blood into a transfusion bag. This autologous blood is often used for trauma or surgical clients with severe blood loss and must be reinfused within 6 hours. The nurse transfuses autologous blood products using the guidelines previously described. Although the client receiving autologous blood is not at risk for most types of transfusion reactions, the nurse must still assess for circulatory overload or bacterial transfusion reactions that can occur as a result of contamination  

 

 

 

 

Von Willebrand Disease in Women

 

Von Willebrand disease is the most common inherited bleeding disorder among American women, with a prevalence of 0.6–1.3% . The overall prevalence is even greater among women with chronic heavy menstrual bleeding, and ranges from 5% to 24%. Among women with heavy menstrual bleeding, von Willebrand disease appears to be more prevalent among Caucasians (15.9%) than African Americans (1.3%).

 

An autosomally inherited congenital bleeding disorder, von Willebrand disease involves a qualitative or quantitative deficiency of von Willebrand factor (vWF), a protein critical for proper platelet adhesion and protection against coagulant factor degradation. Dominant and recessive patterns of inheritance exist. There are three main types of von Willebrand disease. Type 1 (deficiency of vWF), the most common, is usually mild; type 2 (abnormal vWF) has several subtypes and is less common; and type 3 (absence of vWF), which is rare, is the most severe form.

 

Presenting Symptoms and Signs

 

Abnormal uterine bleeding is a commonly reported symptom among women with a diagnosis of von Willebrand disease, with 74–92% experiencing heavy menstrual bleeding. Additional symptoms or signs that may be present include epistaxis (38–63%), gingival bleeding (26–35%), bleeding after dental extraction (29–52%), bleeding from minor cuts or abrasions (36%), postoperative bleeding (20–28%), gastrointestinal bleeding (14%), and joint bleeding (6–8%).

 

Evaluation and Diagnosis

 

Because of the prevalence of von Willebrand disease as well as other inherited and acquired disorders of coagulation and hemostasis in women who seek evaluation for heavy menstrual bleeding, these conditions should be considered in the differential diagnosis of all women who are evaluated for heavy menstrual bleeding, regardless of age. Details on the evaluation and management of women who present with abnormal uterine bleeding are addressed in other publications from the American College of Obstetricians and Gynecologists (the College).

 

The first step in the evaluation of women with suspected bleeding disorders involves obtaining a detailed medical history and performing a physical examination. Women with heavy menstrual bleeding since menarche, postpartum or surgical hemorrhage, plus additional bleeding symptoms, such as bruising, epistaxis, gingival bleeding, or family history of bleeding disorder are considered at risk.

Box 1

includes a screening tool to help clinicians identify adult patients who may benefit from laboratory testing for disorders of hemostasis. Physical examination findings that may suggest a bleeding disorder include petechiae, ecchymoses, or other evidence of recent bleeding, although absence of these signs does not exclude the possibility of an underlying bleeding condition.

 

In patients with a positive screening history, laboratory testing is indicated. An ideal laboratory screening panel to exclude an underlying bleeding disorder is not clearly defined. See Figure 1 for laboratory tests for suspected bleeding disorders. If a patient’s medical history is suggestive of an underlying bleeding condition, specific tests for von Willebrand disease may be indicated, including von Willebrand-ristocetin cofactor activity, vWF antigen, and factor VIII. These test results may be affected by several variables, including stress (eg, surgery, anxiety, or exercise), systemic inflammation or anemia, pregnancy, oral contraceptives, time of the menstrual cycle, sample processing, and the quality of the laboratory. Repeat testing may be necessary to establish a definitive diagnosis. Obtaining a blood specimen for laboratory testing before administering hormonal treatment may be beneficial in some cases. Because existing laboratory assays have limitations and no single diagnostic test reliably identifies von Willebrand disease, it is recommended that these tests be performed and interpreted in conjunction with a hematologist. The platelet function analyzer-100 closure time can be a useful adjunct in screening for disorders of platelet aggregation, but it lacks sensitivity and specificity to be used alone as a screening test. Furthermore, although certain types of von Willebrand disease may be easily distinguished from other bleeding conditions on the basis of laboratory testing, not all types are as straightforward to diagnose. Genetic tests may be necessary for confirmation of certain von Willebrand disease types.

 

Management

 

Once a diagnosis of von Willebrand disease has been established, a multidisciplinary approach to management, which involves obstetrician–gynecologists and hematologists, results in optimal treatment outcomes. Collaboration with a hematologist is recommended to aid in the planning for gynecologic surgery and obstetric management (see “Obstetric Considerations” later in this document). Hematologic consultation can guide decisions related to vWF replacement, optimization of hematologic parameters for epidural anesthesia placement, and use of vWF or factor VIII if necessary for the control of bleeding. Preprocedure vWF, vWF activity, and factor VIII levels may be important in determining the need for and timing of infusion treatment preoperatively and postoperatively. Patients should be reminded that products that prevent platelet adhesion, such as aspirin or nonsteroidal antiinflammatory drugs, should be avoided once von Willebrand disease is diagnosed.

 

Many treatment options are available for women with von Willebrand disease and heavy menstrual bleeding, including hormonal and nonhormonal therapies. This Committee Opinion addresses long-term management of heavy menstrual bleeding in women with bleeding disorders. Management of acute uterine bleeding in women with bleeding disorders is covered elsewhere.

 

Ensuring families have adequate access to care and encouraging the use of medical alert bracelets are also important. Many resources on bleeding disorders exist for patients and health care providers through the National Heart, Lung, and Blood Institute; National Hemophilia Foundation; and the American Society of Hematology.

 

Hormonal Treatments

 

Limited studies have been conducted on the treatment of heavy menstrual bleeding specifically for women with von Willebrand disease or other disorders of hemostasis; the following treatments are based on this evidence and expert opinion. Studies in women with von Willebrand disease and heavy menstrual bleeding suggest that the levonorgestrel-releasing intrauterine system may be effective for this population. Use of progestin-only contraceptives, such as medroxyprogesterone acetate; progestin-only pills; and the progestin implant, also may reduce menstrual flow in the setting of bleeding disorders.

 

For women without bleeding disorders, use of combined oral contraceptives or the levonorgestrel-releasing intrauterine system reduce menstrual bleeding. Limited studies in women with von Willebrand disease and heavy menstrual bleeding suggest these treatments also may be effective for this population. Although oral progestins (taken for 21 days of the cycle) and depot medroxyprogesterone acetate are effective for women with heavy menstrual bleeding without bleeding disorders and may be effective for women with bleeding disorders as well, few studies have focused on their use in this specific population.

 

Nonhormonal Treatments

 

Nonhormonal treatment options include antifibrinolytic agents, such as tranexamic acid and ε-aminocaproic acid, and treatments that increase endogenous plasma concentration of vWF, replace vWF, or promote hemostasis without affecting vWF. Antifibrinolytics inhibit the conversion of plasminogen to plasmin, which inhibits fibrinolysis and, thereby, help stabilize clots. Tranexamic acid was approved for the treatment of heavy menstrual bleeding by the U.S. Food and Drug Administration in 2009. Studies in women without bleeding disorders demonstrate that tranexamic acid reduces menstrual bleeding by 30–55% in women with heavy menstrual bleeding. Theoretically, tranexamic acid also should work in women with von Willebrand disease because it stabilizes clots that have already formed, and clot formation is an essential step in limiting menstrual bleeding.

 

Therapies generally prescribed in conjunction with a hematologist once a diagnosis of von Willebrand disease is established include desmopressin acetate, recombinant factor VIII, and vWF complex infusion. Desmopressin acetate is a synthetic derivative of the antidiuretic hormone vasopressin and works by stimulating the release of vWF from endothelial cells. Recombinant factor VIII and vWF complex infusion are plasma-derived concentrates used to replace factor VIII and vWF, respectively. One study demonstrated that women with bleeding disorders had reduced menstrual flow with the use of either intranasal desmopressin or tranexamic acid.

 

Gynecologic Considerations

 

The association of von Willebrand disease with other gynecologic problems—including ovarian cysts, endometriosis, and leiomyomas––is uncertain. Heavy menstrual bleeding or hemorrhagic ovarian cysts may be managed with combined hormonal contraceptives, which can address both the bleeding and the development of hemorrhagic cysts. For the acute presentation of a ruptured ovarian cyst, patients with von Willebrand disease may require surgical intervention for hemostasis.

 

Obstetric Considerations

 

Obstetric concerns regarding patients with bleeding disorders include spontaneous abortion, mode of delivery, epidural management, operative delivery techniques, and postpartum hemorrhage. Patients with an underlying bleeding disorder are at a high risk of epidural or spinal hematoma. Many experts advocate that women with von Willebrand disease may have a vaginal delivery safely, with cesarean delivery reserved for standard indications. Because von Willebrand disease can be transmitted as an autosomal dominant or recessive trait, the fetus can have up to a 50% risk of being affected. Procedures, such as fetal scalp electrode or fetal scalp sampling, are better avoided, and circumcision should be postponed until the newborn’s vWD status is determined. Operative vaginal deliveries, in which there may be an increased risk of trauma to the newborn, should be avoided because of the potential risk of intracranial hemorrhage.

 

In many women, vWF levels increase in pregnancy and, thus, bleeding risk may be lower than it is when a woman is not pregnant. However, vWF and factor VIII levels are important to assess during pregnancy, including in the third trimester to facilitate planning for delivery and in the event of postpartum hemorrhage. Collaboration with a hematologist is recommended to aid in the planning for delivery because of the risk of hemorrhage. Once estrogen levels begin to decrease in the postpartum period, some individuals with bleeding conditions may present with delayed hemorrhage. Notably, a large epidemiologic study reported that the risk of postpartum hemorrhage for women with von Willebrand disease was 50% higher than for women without a bleeding disorder.

 

Adolescent Considerations

 

The onset of heavy menses at menarche is often the first sign of von Willebrand disease. Among a cohort of 38 women with type 1 von Willebrand disease, retrospective analysis of bleeding symptoms revealed that heavy menstrual bleeding at menarche was the most common initial bleeding symptom, which occurred in 53% of women. However, adolescence is a time when bleeding patterns can be highly variable. If the heavy bleeding is attributed solely to immaturity of the hypothalamic–pituitary axis, an underlying bleeding disorder may be overlooked. Adolescents may have both immaturity of the hypothalamic–pituitary axis and heavy menses, further complicating the presentation. Careful screening is necessary to avoid delay in diagnosis. The College recommends that an initial reproductive health visit occur between the ages of 13 years and 15 years, which provides clinicians with an opportunity to inquire about menstrual history and screen for bleeding disorders. It is particularly important to diagnose bleeding disorders early in children and adolescents because accidental trauma is the most common source of morbidity and mortality in this age group.

 

In adult patients, prior bleeding challenges, such as surgery, dental work, or childbirth, are more common. In adolescents, however, there often is no previous challenge to the hemostatic system. The pictorial bleeding assessment is a tool to specifically record the number of pads or tampons used during the menstrual period, how many times there was passage of clots, and the number of flooding accidents. This tool has been validated in adult women and demonstrates greater than 80% sensitivity and specificity for scores greater than 100 (35). Although this tool may be helpful, clinical utility may be limited. Specific questions can help one gain a better understanding of how heavy an adolescent is bleeding, including the following: How many pads or tampons do you use in a day? How frequently do you need to change your pad or tampon? Do you have flooding? Do you miss school because of your period? A simple screening tool, which consists of a set of eight questions that focus on bleeding history, is equally sensitive to the pictorial bleeding assessment. The combination of a pictorial bleeding assessment score greater than 185 and at least one question with a positive result on the screening questionnaire increased the sensitivity to 95% for the diagnosis of an underlying bleeding disorder and 91% for von Willebrand disease; the positive predictive value was 71% and 5%, respectively. Although several screening questionnaires exist to help identify women with heavy menstrual bleeding who need further hemostatic evaluation, the screening tool in

Box 2

may be more appropriate for adolescent patients. If screening results are positive, laboratory evaluation is indicated. Screening for disorders of hemostasis before starting hormonal treatment is recommended because treatment may interfere with the results and combined oral contraceptive treatment should be interrupted for accurate von Willebrand disease testing. Although recent studies have suggested that interrupting combined hormonal contraceptive therapy for von Willebrand testing may be unnecessary (37), further research in this area is needed. Given emerging controversy surrounding von Willebrand disease testing, consultation with a hematologist may be helpful for the management of patients already taking combined hormonal contraceptives.

 

Available treatment options for adolescents are similar to those for other women. In adolescents, fertility preservation is paramount; therefore, medical options should be used rather than surgical procedures. Antifibrinolytic agents, such as tranexamic acid and ε-aminocaproic acid, can be effective nonhormonal treatment options for adolescents; however, their use in individuals younger than 18 years is considered “off-label.” As with the adult patient, a multidisciplinary approach to management, which involves both obstetrician–gynecologists and hematologists, results in optimal treatment outcomes.

 

Conclusion

 

Von Willebrand disease is a common cause of heavy menstrual bleeding and other bleeding problems in women and adolescent girls. Obstetrician–gynecologists should include von Willebrand disease and other bleeding disorders in the differential diagnosis when evaluating patients with heavy menstrual bleeding, regardless of age. Once a diagnosis is established, collaboration with a hematologist is recommended for the long-term care of patients with bleeding disorders, such as von Willebrand disease. Many resources exist for patients and health care providers through the National Heart, Lung, and Blood Institute; National Hemophilia Foundation; and the American Society of Hematology.