Interventions for Clients with Hematologic Problems RED BLOOD CELL DISORDERS

Interventions for Clients Interventions for Clients with Hematologic with Hematologic

ProblemsProblems

RED BLOOD CELL DISORDERSRED BLOOD CELL DISORDERS

Disorders of the hematologic system Disorders of the hematologic system can occur as a result of problems in can occur as a result of problems in the production, function, or normal the production, function, or normal destruction of any type of blood cell. destruction of any type of blood cell.

The type and severity of the specific The type and severity of the specific disorder determine the degree of disorder determine the degree of threat to the client's well-beingthreat to the client's well-being

RED BLOOD CELL DISORDERSRED BLOOD CELL DISORDERS

The major cellular population of the blood The major cellular population of the blood consists of red blood cells (RBCs), or erythrocytes. consists of red blood cells (RBCs), or erythrocytes.

Adequate tissue oxygenation depends on Adequate tissue oxygenation depends on maintaining the circulating number of RBCs maintaining the circulating number of RBCs within the normal range for the person's age and within the normal range for the person's age and gender and ensuring that the cells can perform gender and ensuring that the cells can perform their normal functions. RBC disorders include their normal functions. RBC disorders include problems in production, function, andproblems in production, function, and destruction. destruction.

These problems may result in an insufficient These problems may result in an insufficient number or insufficient function of RBCs number or insufficient function of RBCs (anemia) (anemia) or an excess of RBCs or an excess of RBCs (polycythemia).(polycythemia).

RED BLOOD CELL RED BLOOD CELL DISORDERSDISORDERS

Anemia is a reduction in either the number of Anemia is a reduction in either the number of RBCs, the quantity of hemoglobin, or the RBCs, the quantity of hemoglobin, or the hematocrit (percentage of packed RBCs per hematocrit (percentage of packed RBCs per deciliter of blood). deciliter of blood).

Anemia is a clinical sign, not a diagnosis, because Anemia is a clinical sign, not a diagnosis, because it is a manifestation of a number of abnormal it is a manifestation of a number of abnormal conditions.conditions.

Despite the many causes of anemia, the effects Despite the many causes of anemia, the effects of anemia on the client and the corresponding of anemia on the client and the corresponding nursing care are similar for all types of anemianursing care are similar for all types of anemia

ANEMIAS RESULTING FROM INCREASED ANEMIAS RESULTING FROM INCREASED DESTRUCTION OF RED BLOOD CELLSDESTRUCTION OF RED BLOOD CELLS

Sickle Cell DiseaseSickle Cell Disease

Sickle cell disease is a condition in which chronic Sickle cell disease is a condition in which chronic anemia is one of many problems causing pain, anemia is one of many problems causing pain, disability, increased risk for disease, and early disability, increased risk for disease, and early death. death.

Once considered a childhood disorder, clients Once considered a childhood disorder, clients with sickle cell disease who receive appropriate with sickle cell disease who receive appropriate supportive care may live into their 30s and 40s. supportive care may live into their 30s and 40s.

In addition, there is great variation among clients In addition, there is great variation among clients in the severity of the disease and the onset of in the severity of the disease and the onset of complications.complications.



PathophysiologyPathophysiology Hereditary disorder - formation of abnormal beta chains in Hereditary disorder - formation of abnormal beta chains in

the hemoglobin molecule. the hemoglobin molecule.

TheThe normal hemoglobin molecule of adults is composed normal hemoglobin molecule of adults is composed partially of the globin protein, consisting of two alpha partially of the globin protein, consisting of two alpha chains and two beta chains of amino acids (chains and two beta chains of amino acids (hemoglobin hemoglobin A A (HbA)). (HbA)). The total hemoglobin of normal healthy adults is The total hemoglobin of normal healthy adults is usually 98% to 99% HbA, with a small percentage of a fetal usually 98% to 99% HbA, with a small percentage of a fetal form of hemoglobin (HbF).form of hemoglobin (HbF).

In sickle cell disease, at least 40% of the total hemoglobin In sickle cell disease, at least 40% of the total hemoglobin contains an abnormality of the beta chains, known as contains an abnormality of the beta chains, known as hemoglobin S (HbS). hemoglobin S (HbS).


Sickle Cell DiseaseSickle Cell Disease HbS is sensitive to changes in the oxygen content of the HbS is sensitive to changes in the oxygen content of the

RBC. When RBCs containing large amounts of HbS are RBC. When RBCs containing large amounts of HbS are exposed to conditions of decreased oxygen, the abnormal exposed to conditions of decreased oxygen, the abnormal beta chains contract and pile together within the cell, beta chains contract and pile together within the cell, distorting the overall shape of the RBC. distorting the overall shape of the RBC.

These cells assume a sickle shape, become rigid, clump These cells assume a sickle shape, become rigid, clump together, and form clusters that block capillary blood flow. together, and form clusters that block capillary blood flow.

Capillary obstruction leads to further tissue Capillary obstruction leads to further tissue hypoxia hypoxia (reduced oxygen supply) and more sickling, causing blood (reduced oxygen supply) and more sickling, causing blood vessel obstructions and infarctions in the locally affected vessel obstructions and infarctions in the locally affected tissues. tissues.

Situations that lead to sickling include hypoxia, dehydration, Situations that lead to sickling include hypoxia, dehydration, infections, vascular stasis, low environmental or body infections, vascular stasis, low environmental or body temperatures, acidosis, strenuous exercise, and anesthesiatemperatures, acidosis, strenuous exercise, and anesthesia

Red blood cell actions under conditions of low tissue oxygenation. (HbS, Hemoglobin S; HbA, hemoglobin A.)


Sickle Cell DiseaseSickle Cell Disease Usually, sickled cells resume a normal shape when Usually, sickled cells resume a normal shape when

the precipitating condition is removed and proper the precipitating condition is removed and proper oxygenation occurs. oxygenation occurs.

The membranes of the cells become damaged over The membranes of the cells become damaged over time, and cells become irreversibly sickled. time, and cells become irreversibly sickled.

The membranes of cells with HbS are more fragile The membranes of cells with HbS are more fragile and more easily destroyed in the spleen and in and more easily destroyed in the spleen and in other organs that have long, twisted capillary other organs that have long, twisted capillary pathways. The average life span of an RBC pathways. The average life span of an RBC containing 40% or more of HbS is approximately 20 containing 40% or more of HbS is approximately 20 days. This reduced life span is responsible for days. This reduced life span is responsible for hemolytic hemolytic (blood cell-destroying) anemia in (blood cell-destroying) anemia in clients with sickle cell disease.clients with sickle cell disease.


Sickle Cell DiseaseSickle Cell Disease The client with sickle cell disease experiences periodic The client with sickle cell disease experiences periodic

episodes of extensive cellular sickling, or episodes of extensive cellular sickling, or crises. crises.

Repeated occlusions of progressively larger blood vessels Repeated occlusions of progressively larger blood vessels have long-term negative effects on tissues and organs. have long-term negative effects on tissues and organs. Most effects are thought to occur as a result of capillary and Most effects are thought to occur as a result of capillary and blood vessel occlusion leading to tissue hypoxia, anoxia, blood vessel occlusion leading to tissue hypoxia, anoxia, ischemia, and cell death.ischemia, and cell death.

Tissues and organs begin toTissues and organs begin to have small infarcted areas that have small infarcted areas that eventually destroy all healthy cells and lead to organ eventually destroy all healthy cells and lead to organ failure. failure.

Tissues and organs most commonly affected in this way are Tissues and organs most commonly affected in this way are the spleen, liver, heart, kidney, brain, bones, and retinathe spleen, liver, heart, kidney, brain, bones, and retina



EtiologyEtiology Sickle cell disease is a genetic disorder with an autosomal Sickle cell disease is a genetic disorder with an autosomal

recessive pattern of inheritance. recessive pattern of inheritance.

The formation of the beta chains of the hemoglobin The formation of the beta chains of the hemoglobin molecule is dependent on a pair of genes. molecule is dependent on a pair of genes.

When the client inherits one abnormal gene of this pair, the When the client inherits one abnormal gene of this pair, the condition is called condition is called sickle cell trait. sickle cell trait.

When the client inherits two abnormal genes, the condition When the client inherits two abnormal genes, the condition is called is called sickle cell disease sickle cell disease (formerly sickle cell anemia), (formerly sickle cell anemia), and the client has severe manifestations of the disease and the client has severe manifestations of the disease even under relatively mild precipitating conditions. In even under relatively mild precipitating conditions. In addition, if the client has children, each child will inherit one addition, if the client has children, each child will inherit one of the two abnormal genes and at least have sickle cell traitof the two abnormal genes and at least have sickle cell trait



Cultural considerationsCultural considerations Sickle cell disease occurs most often in African Sickle cell disease occurs most often in African

Americans, as well as in African, Mediterranean, Americans, as well as in African, Mediterranean, Caribbean, Middle Eastern, and Central American Caribbean, Middle Eastern, and Central American populations. populations.

Approximately 1 of every 12 African Americans Approximately 1 of every 12 African Americans has the sickle cell trait. has the sickle cell trait.

One of every 345 African-American infants One of every 345 African-American infants inherits two abnormal genes (one from each inherits two abnormal genes (one from each parent) and has overt sickle cell disease parent) and has overt sickle cell disease



HistoryHistory An adult with sickle cell disease has a long-standing An adult with sickle cell disease has a long-standing

diagnosis of the disorder. diagnosis of the disorder. The nurse asks the client about previous crises, including The nurse asks the client about previous crises, including

precipitating events, severity, and usual treatments. precipitating events, severity, and usual treatments. Recent ac tivities and situations are explored to determine Recent ac tivities and situations are explored to determine

the probable precipitating condition or event. the probable precipitating condition or event. The nurse also reviews all activities and events during the The nurse also reviews all activities and events during the

previous 24 hours, including food and fluid intake, exposure previous 24 hours, including food and fluid intake, exposure to temperature extremes, types of clothing worn, to temperature extremes, types of clothing worn, medications taken, exercise, trauma, stress, and ingestion medications taken, exercise, trauma, stress, and ingestion of alcohol or other recreational drugs. This activity review of alcohol or other recreational drugs. This activity review provides important information about fatigue, activity provides important information about fatigue, activity tolerance, and participation in activities of daily living tolerance, and participation in activities of daily living (ADLs).(ADLs).



The client is asked about changes in sleep The client is asked about changes in sleep and rest patterns, ability to climb stairs, and and rest patterns, ability to climb stairs, and any activity that induces shortness of breath. any activity that induces shortness of breath.

Obtaining a subjective baseline assessment Obtaining a subjective baseline assessment of the client's perceived energy level using a of the client's perceived energy level using a scale ranging from 0 to 10 (0 = not tired with scale ranging from 0 to 10 (0 = not tired with plenty of energy; 10 = total exhaustion) can plenty of energy; 10 = total exhaustion) can be useful in evaluating the degree of fatigue be useful in evaluating the degree of fatigue and the effectiveness of later treatmentsand the effectiveness of later treatments



Physical assessment/clinical manifestationsPhysical assessment/clinical manifestations Pain is the most common symptom experienced during sickle Pain is the most common symptom experienced during sickle

cell crisis. cell crisis. Jaundice may also be present as a result of increased red Jaundice may also be present as a result of increased red

blood cell (RBC) destruction and release of bilirubin. blood cell (RBC) destruction and release of bilirubin. Other clinical manifestations vary with the site of tissue Other clinical manifestations vary with the site of tissue

damage.damage.

Cardiovascular assessmentCardiovascular assessment Compare peripheral pulses, temperature, and capillary refill Compare peripheral pulses, temperature, and capillary refill

in all extremitiesin all extremities Extremities distal to blood vessel occlusion are cool to the Extremities distal to blood vessel occlusion are cool to the

touch with slow capillary refill and may have diminished or touch with slow capillary refill and may have diminished or absent pulses. absent pulses.

The heart rate may be rapid and the blood pressure low to The heart rate may be rapid and the blood pressure low to average, with a decreased pulse pressure because breakage average, with a decreased pulse pressure because breakage of RBCs leads to anemiaof RBCs leads to anemia



Integumentary assessment Integumentary assessment The skin may be pale or cyanotic as a result of decreased The skin may be pale or cyanotic as a result of decreased

perfusion and anemia. The nurse examines the lips, tongue, perfusion and anemia. The nurse examines the lips, tongue, nail beds, conjunctivae, palms, and soles at regular nail beds, conjunctivae, palms, and soles at regular intervals for subtle color changes. With cyanosis, the lips intervals for subtle color changes. With cyanosis, the lips and tongue are gray, and the palms, soles, conjunctivae, and tongue are gray, and the palms, soles, conjunctivae, and nail beds have a bluish tinge.and nail beds have a bluish tinge.

Jaundice. The nurse assesses for jaundice in clients with Jaundice. The nurse assesses for jaundice in clients with darker skin by inspecting the oral mucosa, especially the darker skin by inspecting the oral mucosa, especially the hard palate, for yellow discoloration. Inspection of the hard palate, for yellow discoloration. Inspection of the conjunctivae and adjacent sclera may be misleading conjunctivae and adjacent sclera may be misleading because of normal deposits of subconjunctival fat that because of normal deposits of subconjunctival fat that produce a yellowish hue when seen in contrast to the dark produce a yellowish hue when seen in contrast to the dark periorbital skin. Therefore the nurse examines the sclera periorbital skin. Therefore the nurse examines the sclera closest to the cornea to diagnose jaundice more accurately. closest to the cornea to diagnose jaundice more accurately. Jaundice from excessive bilirubin may also cause intense Jaundice from excessive bilirubin may also cause intense itching.itching.



Abdominal assessmentAbdominal assessment Abdominal organs are usually the first to be damaged as a Abdominal organs are usually the first to be damaged as a

result of multiple episodes of hypoxia and ischemia. The result of multiple episodes of hypoxia and ischemia. The nurse inspects the abdomen for asymmetry or bulging nurse inspects the abdomen for asymmetry or bulging areas, gently palpating it. Affected organs, such as the liver areas, gently palpating it. Affected organs, such as the liver or spleen, may be firm and enlarged with a nodular texture or spleen, may be firm and enlarged with a nodular texture in later stages of the diseasein later stages of the disease

Musculoskeletal assessmentMusculoskeletal assessment Extremities are a common site of vascular occlusion among Extremities are a common site of vascular occlusion among

clients who have sickle cell disease. In addition, joints may clients who have sickle cell disease. In addition, joints may be damaged from frequent hypoxic episodes and undergo be damaged from frequent hypoxic episodes and undergo necrotic degeneration. necrotic degeneration.

The nurse inspects the extremities for symmetry and The nurse inspects the extremities for symmetry and records any areas of swelling or color difference. Clients are records any areas of swelling or color difference. Clients are asked to move all joints, and the nurse notes the range of asked to move all joints, and the nurse notes the range of motion and any accompanying painmotion and any accompanying pain


Sickle Cell DiseaseSickle Cell DiseaseCentral nervous system assessmentCentral nervous system assessment During crises, clients may have a low-grade fever. If the CNS During crises, clients may have a low-grade fever. If the CNS

sustains infarcts or repeated episodes of hypoxia, they may have sustains infarcts or repeated episodes of hypoxia, they may have seizure activity or clinical manifestations of a stroke. Hand grasps seizure activity or clinical manifestations of a stroke. Hand grasps are assessed bilaterally. The nurse assesses gait and coordination are assessed bilaterally. The nurse assesses gait and coordination in those clients who are permitted to walk.in those clients who are permitted to walk.

Laboratory assessmentLaboratory assessment Large percentage of hemoglobin S (HbS) present on Large percentage of hemoglobin S (HbS) present on

electrophoresis. A person who has sickle cell trait usually electrophoresis. A person who has sickle cell trait usually expresses less than 40% HbS, and the client with sickle cell expresses less than 40% HbS, and the client with sickle cell disease may express 85% to 95% HbS. disease may express 85% to 95% HbS. This percentage does not This percentage does not change during crises. change during crises.

Another indicator of sickle cell disease is the percentage of RBCs Another indicator of sickle cell disease is the percentage of RBCs showing irreversible sickling. This value is less than 1% among showing irreversible sickling. This value is less than 1% among people who do not have sickle cell disease, is 5% to 50% among people who do not have sickle cell disease, is 5% to 50% among people with sickle cell trait, and may exceed 90% among clients people with sickle cell trait, and may exceed 90% among clients with sickle cell diseasewith sickle cell disease


Sickle Cell DiseaseSickle Cell Disease The hematocrit of clients with sickle cell disease is usually The hematocrit of clients with sickle cell disease is usually

low (between 20% and 30%). This value decreases even low (between 20% and 30%). This value decreases even more dramatically during vascular occlusive crises, or more dramatically during vascular occlusive crises, or aplastic crises, when the bone marrow temporarily fails to aplastic crises, when the bone marrow temporarily fails to produce cells during stressful periods (such as infection). produce cells during stressful periods (such as infection).

The reticulocyte count is elevated, indicating anemia of The reticulocyte count is elevated, indicating anemia of long duration. Often the mean corpuscular hemoglobin long duration. Often the mean corpuscular hemoglobin concentration (MCHC) and total bilirubin level are elevated concentration (MCHC) and total bilirubin level are elevated in the client who has sickle cell disease.in the client who has sickle cell disease.

The total white blood cell (WBC) count is usually above The total white blood cell (WBC) count is usually above normal among clients who have sickle cell disease. It is normal among clients who have sickle cell disease. It is thought that this elevation is related to chronic thought that this elevation is related to chronic inflammation resulting from tissue hypoxia and ischemiainflammation resulting from tissue hypoxia and ischemia



Radiographic assessmentRadiographic assessment Bone changes occur as a result of chronically stimulated Bone changes occur as a result of chronically stimulated

marrow and hypoxic bone tissue. The skull may show marrow and hypoxic bone tissue. The skull may show radiographic changes resulting from chronic bone surface radiographic changes resulting from chronic bone surface resorption and regeneration, giving the skull a "crew cut" resorption and regeneration, giving the skull a "crew cut" appearance. Joint necrosis and degeneration also are appearance. Joint necrosis and degeneration also are obvious on x-ray examination.obvious on x-ray examination.

Other diagnostic assessmentOther diagnostic assessment Electrocardiographic (ECG) changes document cardiac Electrocardiographic (ECG) changes document cardiac

infarcts and tissue damage. infarcts and tissue damage. Ultra-sonography, computed tomography (CT), positron Ultra-sonography, computed tomography (CT), positron

emission tomography (PET), and magnetic resonance emission tomography (PET), and magnetic resonance imaging (MRI) may reveal soft-tissue and organ imaging (MRI) may reveal soft-tissue and organ degenerative changes resulting from inadequate degenerative changes resulting from inadequate oxygenation and chronic inflammationoxygenation and chronic inflammation



InterventionsInterventions PAIN DRUG THERAPY. Clients in acute sickle cell crisis often PAIN DRUG THERAPY. Clients in acute sickle cell crisis often

require at least 48 hours of parenteral analgesics. require at least 48 hours of parenteral analgesics. Morphine and hydromorphone (Dilaudid) are the medications Morphine and hydromorphone (Dilaudid) are the medications

of choiceof choice For sickle cell crisis, these agents should be administered For sickle cell crisis, these agents should be administered

intravenously on a routine schedule. Once relief is obtained, intravenously on a routine schedule. Once relief is obtained, the intravenous (IV) dose can be tapered and then the intravenous (IV) dose can be tapered and then administered orallyadministered orally

Meperidine (Demerol) is also used for sickle cell crisis, but Meperidine (Demerol) is also used for sickle cell crisis, but long-term use of this agent can cause neurologic symptoms, long-term use of this agent can cause neurologic symptoms, including anxiety and seizuresincluding anxiety and seizures

Intramuscular (IM) injections are avoidedIntramuscular (IM) injections are avoided because frequent because frequent injections lead to sclerosing of tissue (and absorption may be injections lead to sclerosing of tissue (and absorption may be impaired by poor circulation). impaired by poor circulation).

Moderate pain may be treated with oral doses of codeine, Moderate pain may be treated with oral doses of codeine, morphine sulfate, or nonsteroidal antiinflammatory drugs morphine sulfate, or nonsteroidal antiinflammatory drugs (NSAIDs)(NSAIDs)


Sickle Cell DiseaseSickle Cell Disease Complementary therapies and other Complementary therapies and other

nonpharmacologic measures, such as nonpharmacologic measures, such as keeping the room warm, using distraction keeping the room warm, using distraction and relaxation techniques, proper and relaxation techniques, proper positioning with support for painful areas, positioning with support for painful areas, aroma therapy, therapeutic touch, and aroma therapy, therapeutic touch, and warm soaks or compresses, have all been warm soaks or compresses, have all been useful in decreasing pain. useful in decreasing pain.

The nurse must not assume, however, that The nurse must not assume, however, that these methods alone will provide adequate these methods alone will provide adequate pain relief. Analgesics are required to treat pain relief. Analgesics are required to treat sickle cell painsickle cell pain


Sickle Cell DiseaseSickle Cell Disease POTENTIAL FOR SEPSIS. The client with sickle cell disease is more POTENTIAL FOR SEPSIS. The client with sickle cell disease is more

susceptible to bloodborne infections and infection by encapsulated susceptible to bloodborne infections and infection by encapsulated microorganisms, such as microorganisms, such as Streptococcus pneumoniae Streptococcus pneumoniae and and Haemophilus influenzae, Haemophilus influenzae, as a result of decreased spleen function. as a result of decreased spleen function. Interventions aim at preventing or halting the process of infection, Interventions aim at preventing or halting the process of infection, controlling infection, and initiating early, effective treatment controlling infection, and initiating early, effective treatment regimens for specific infections.regimens for specific infections.

PREVENTION/EARLY DETECTION.PREVENTION/EARLY DETECTION. Frequent, thorough handwashing is Frequent, thorough handwashing is of the utmost importance. Any person with an upper respiratory tract of the utmost importance. Any person with an upper respiratory tract infection who must enter the client's room wears a mask. Strict infection who must enter the client's room wears a mask. Strict aseptic technique is used for all invasive procedures.aseptic technique is used for all invasive procedures.

The nurse continually assesses the client for the presence of The nurse continually assesses the client for the presence of infection and monitors a daily complete blood count (CBC) with infection and monitors a daily complete blood count (CBC) with differential WBC count. The oral mucosa is inspected during every differential WBC count. The oral mucosa is inspected during every nursing shift for lesions indicating fungal or viral infection. The lungs nursing shift for lesions indicating fungal or viral infection. The lungs are auscultated every 8 hours for crackles, wheezes, or diminished are auscultated every 8 hours for crackles, wheezes, or diminished breath sounds. Each time the client voids, assistive nursing breath sounds. Each time the client voids, assistive nursing personnel inspect the urine for odor and cloudiness, and the client is personnel inspect the urine for odor and cloudiness, and the client is asked about any sen sation of urgency, burning, or pain during asked about any sen sation of urgency, burning, or pain during urination. Vital signs are taken at least every 4 hours to assess for urination. Vital signs are taken at least every 4 hours to assess for feverfever



DRUG THERAPY.DRUG THERAPY. Prophylactic therapy Prophylactic therapy with twice-daily administration of oral with twice-daily administration of oral penicillin in the penicillin-tolerant client penicillin in the penicillin-tolerant client has resulted in dramatic reductions in has resulted in dramatic reductions in the number of pneumonia and other the number of pneumonia and other streptococcal infections. Agents used streptococcal infections. Agents used depend on the sensitivity of the specific depend on the sensitivity of the specific organism causing the infection, as well organism causing the infection, as well as the extent of the infectionas the extent of the infection


Sickle Cell DiseaseSickle Cell Disease POTENTIAL FOR MULTIPLE ORGAN DYSFUNCTIONPOTENTIAL FOR MULTIPLE ORGAN DYSFUNCTION The client in sickle cell crisis is admitted to the acute care hospital. The nurse The client in sickle cell crisis is admitted to the acute care hospital. The nurse

assesses for adequacy of circulation to all body areas. Restrictive clothing is assesses for adequacy of circulation to all body areas. Restrictive clothing is removed, and the client is in structed to avoid keeping the hips or knees in a removed, and the client is in structed to avoid keeping the hips or knees in a flexed position.flexed position.

Dehydration perpetuates cell sickling and must be avoided. Nursing Dehydration perpetuates cell sickling and must be avoided. Nursing personnel assist the client in maintaining an adequate hydration status. The personnel assist the client in maintaining an adequate hydration status. The client in crisis requires an oral or parenteral intake of at least 200 mL/hr.client in crisis requires an oral or parenteral intake of at least 200 mL/hr.

Oxygen is ordered, and the nurse ensures that oxygen therapy is delivered Oxygen is ordered, and the nurse ensures that oxygen therapy is delivered appropriately, including nebulization to prevent dehydration. appropriately, including nebulization to prevent dehydration.

Transfusion therapy has been used to decrease the incidence of organ Transfusion therapy has been used to decrease the incidence of organ dysfunction and stroke. RBC transfusions are therapeutic because levels of dysfunction and stroke. RBC transfusions are therapeutic because levels of hemoglobin A (HbA) are sustained, whereas levels of hemoglobin S (HbS) are hemoglobin A (HbA) are sustained, whereas levels of hemoglobin S (HbS) are diluted. Transfusions also suppress erythropoiesis, thereby decreasing the diluted. Transfusions also suppress erythropoiesis, thereby decreasing the production of sickle cells. Transfusions may be administered in either the production of sickle cells. Transfusions may be administered in either the acute care or clinic setting by a registered nurse. The nurse monitors the acute care or clinic setting by a registered nurse. The nurse monitors the client closely for complications of transfusion therapyclient closely for complications of transfusion therapy

In some treatment centers, bone marrow transplantation is being performed In some treatment centers, bone marrow transplantation is being performed to correct abnormal hemoglobin permanently. Because bone marrow to correct abnormal hemoglobin permanently. Because bone marrow transplantation is expensive and may result in chronic and life-threatening transplantation is expensive and may result in chronic and life-threatening complications, its risks and benefits need to be seriously considered for each complications, its risks and benefits need to be seriously considered for each clientclient


Glucose-6-Phosphate Dehydrogenase Deficiency Glucose-6-Phosphate Dehydrogenase Deficiency AnemiaAnemia

OverviewOverview Many forms of congenital Many forms of congenital hemolytic hemolytic (blood cell-destroying) anemia (blood cell-destroying) anemia

result from defects or deficiencies of one or more enzymes within the result from defects or deficiencies of one or more enzymes within the red blood cell (RBC). More than 200 such disorders have been identified. red blood cell (RBC). More than 200 such disorders have been identified. Most of these enzymes are needed to complete some critical step in Most of these enzymes are needed to complete some critical step in cellular energy production. The most common type of congenital cellular energy production. The most common type of congenital hemolytic anemia is associated with a deficiency of the enzyme hemolytic anemia is associated with a deficiency of the enzyme glucose-6-phosphate dehydrogenase (G6PD). This disease is inherited glucose-6-phosphate dehydrogenase (G6PD). This disease is inherited as an X-linked recessive disorder and affects about 10% of all African as an X-linked recessive disorder and affects about 10% of all African Americans.Americans.

G6PD stimulates critical reactions in the glycolytic pathway. RBCs G6PD stimulates critical reactions in the glycolytic pathway. RBCs contain no contain no mitochondria, mitochondria, so active glycolysis is essential for energy so active glycolysis is essential for energy metabolism. Newly produced RBCs from clients with G6PD deficiency metabolism. Newly produced RBCs from clients with G6PD deficiency have relatively sufficient quantities of G6PD; however, as the cells age, have relatively sufficient quantities of G6PD; however, as the cells age, the concentration diminishes drastically. Cells that have reduced the concentration diminishes drastically. Cells that have reduced amounts of G6PD are more susceptible to breaking during exposure to amounts of G6PD are more susceptible to breaking during exposure to specific drugs (e.g., phenacetin, sulfonamides, aspirin [acetylsalicylic specific drugs (e.g., phenacetin, sulfonamides, aspirin [acetylsalicylic acid], quinine derivatives, thiazide diuretics, and vitamin K derivatives) acid], quinine derivatives, thiazide diuretics, and vitamin K derivatives) and toxins.and toxins.

After exposure to any of these agents, clients experience acute After exposure to any of these agents, clients experience acute intravascular hemolysis lasting from 7 to 12 days. During this acute intravascular hemolysis lasting from 7 to 12 days. During this acute phase, anemia and jaundice develop. The hemolytic reaction is self-phase, anemia and jaundice develop. The hemolytic reaction is self-limited because only older erythrocytes, containing less G6PD, are limited because only older erythrocytes, containing less G6PD, are destroyeddestroyed


Glucose-6-Phosphate Dehydrogenase Deficiency Glucose-6-Phosphate Dehydrogenase Deficiency AnemiaAnemia

Collaborative managementCollaborative management It is critical that the precipitating drug or the agent It is critical that the precipitating drug or the agent

responsible for the hemolytic reaction be identified and responsible for the hemolytic reaction be identified and totally removed. People should be screened for this totally removed. People should be screened for this deficiency before donating blood, because administration of deficiency before donating blood, because administration of cells deficient in G6PD can be hazardous for the recipient.cells deficient in G6PD can be hazardous for the recipient.

During and immediately after an episode of hemolysis, During and immediately after an episode of hemolysis, adequate hydration is essential to prevent precipitation of adequate hydration is essential to prevent precipitation of cellular debris and hemoglobin in the kidney tubules, which cellular debris and hemoglobin in the kidney tubules, which can lead to acute tubular necrosis. Osmotic diuretics, such can lead to acute tubular necrosis. Osmotic diuretics, such as mannitol (Osmitrol), may assist in preventing this as mannitol (Osmitrol), may assist in preventing this complication. Transfusion therapy is indicated when anemia complication. Transfusion therapy is indicated when anemia is present and kidney function is normalis present and kidney function is normal


Immunohemolytic AnemiaImmunohemolytic Anemia

OverviewOverview Increased RBC destruction through hemolysis can occur in Increased RBC destruction through hemolysis can occur in

response to many situations, including trauma, infection response to many situations, including trauma, infection (especially malarial infections), and autoimmune reactions. (especially malarial infections), and autoimmune reactions. All increase the rate at which RBCs are destroyed by All increase the rate at which RBCs are destroyed by causing lysis (breakage) of the RBC membrane. causing lysis (breakage) of the RBC membrane.

In immunohemolytic anemia, immune system components In immunohemolytic anemia, immune system components attack a person's own RBCs. The exact mechanism that attack a person's own RBCs. The exact mechanism that causes immune components to no longer recognize blood causes immune components to no longer recognize blood cells as self and to initiate destructive processes against cells as self and to initiate destructive processes against RBCs is not known. Some hemolytic anemias are present RBCs is not known. Some hemolytic anemias are present with other autoimmune disorders (such as systemic lupus with other autoimmune disorders (such as systemic lupus erythematosus) or lymphoproliferative disorders. erythematosus) or lymphoproliferative disorders. Regardless of the cause, RBCs are viewed as non-self by Regardless of the cause, RBCs are viewed as non-self by the immune system and are destroyed.the immune system and are destroyed.



There are two types of immunohemolytic anemia: warm There are two types of immunohemolytic anemia: warm antibody anemia and cold antibody anemia. antibody anemia and cold antibody anemia.

Warm antibody anemia Warm antibody anemia is usually associated with is usually associated with immunoglobulin G (IgG) antibody excess. These antibodies immunoglobulin G (IgG) antibody excess. These antibodies are most active at 98° F (37° C) and may be stimulated by are most active at 98° F (37° C) and may be stimulated by drugs, chemicals, or other autoimmune problems. drugs, chemicals, or other autoimmune problems.

Cold antibody anemia Cold antibody anemia is associated with fixation of is associated with fixation of complement proteins on immunoglobulin Mcomplement proteins on immunoglobulin M (IgM) and (IgM) and occurs best at 86° F (30° C). This problem is commonly occurs best at 86° F (30° C). This problem is commonly associated with a Raynaud-like response in which the associated with a Raynaud-like response in which the arteries in the distal extremities constrict profoundly in arteries in the distal extremities constrict profoundly in response to cold temperatures or stressresponse to cold temperatures or stress



Collaborative managementCollaborative management Treatment depends on clinical severity. Steroid Treatment depends on clinical severity. Steroid

therapy for mild to moderate immunosuppression therapy for mild to moderate immunosuppression is the first line of treatment and is temporarily is the first line of treatment and is temporarily effective in most clients. Splenectomy and more effective in most clients. Splenectomy and more intensive immunosuppressive therapy with intensive immunosuppressive therapy with cyclophosphamide (Cytoxan, Procytox) and cyclophosphamide (Cytoxan, Procytox) and azathioprine (Imuran) may be instituted if steroid azathioprine (Imuran) may be instituted if steroid therapy fails. Plasma exchange therapy to therapy fails. Plasma exchange therapy to remove attacking antibodies is effective for remove attacking antibodies is effective for clients who do not respond to immunosuppressive clients who do not respond to immunosuppressive therapytherapy

Indications for treatment with blood components

ANEMIAS RESULTING FROM DECREASEDPRODUCTION OF RED BLOOD CELLS

Iron Deficiency Anemia

OverviewOverview The adult body contains between 2 and 6 g of iron, depending The adult body contains between 2 and 6 g of iron, depending

on the size of the person and the amount of hemoglobin in on the size of the person and the amount of hemoglobin in the cells. the cells.

Approximately two thirds of this iron is contained in Approximately two thirds of this iron is contained in hemoglobin; the other third is stored in the bone marrow, hemoglobin; the other third is stored in the bone marrow, spleen, liver, and muscle. spleen, liver, and muscle.

If a person has an iron deficiency, the iron stores are depleted If a person has an iron deficiency, the iron stores are depleted first, followed by the hemoglobin stores. As a result, RBCs are first, followed by the hemoglobin stores. As a result, RBCs are small small (microcytic), (microcytic), and the client has relatively mild and the client has relatively mild manifestations of anemia, including weakness and pallor. In manifestations of anemia, including weakness and pallor. In iron deficiency anemia, serum ferritin values are less than 12 iron deficiency anemia, serum ferritin values are less than 12 g/L.g/L.

Iron deficiency anemia is the most common type of anemia Iron deficiency anemia is the most common type of anemia and can result from blood loss, increased energy demands, and can result from blood loss, increased energy demands, gastrointestinal malabsorption, and dietary inadequacy. gastrointestinal malabsorption, and dietary inadequacy.

The basic problem of iron deficiency anemia is a decreased The basic problem of iron deficiency anemia is a decreased supply of iron for the developing RBC. Iron deficiency anemia supply of iron for the developing RBC. Iron deficiency anemia can occur at any age but is more frequent in women, older can occur at any age but is more frequent in women, older adults, and people with poor dietsadults, and people with poor diets


Iron Deficiency Anemia

Collaborative managementCollaborative management The primary treatment of clients with iron deficiency anemia The primary treatment of clients with iron deficiency anemia

is to increase the oral intake of iron from common food is to increase the oral intake of iron from common food sources. sources.

An adequate diet supplies a person with about 10 to 15 mg An adequate diet supplies a person with about 10 to 15 mg of iron per day, of which only 5% to 10% is absorbed in the of iron per day, of which only 5% to 10% is absorbed in the stomach, duodenum, and upper jejunum. This amount is stomach, duodenum, and upper jejunum. This amount is sufficient to meet the needs of healthy men and healthy sufficient to meet the needs of healthy men and healthy women after childbearing age but is not sufficient to supply women after childbearing age but is not sufficient to supply the greater needs of menstruating women and adolescents the greater needs of menstruating women and adolescents during growth spurts. during growth spurts.

Fortunately, if iron intake is inadequate, or if bleeding or Fortunately, if iron intake is inadequate, or if bleeding or pregnancy occurs, the gastrointestinal tract is capable of pregnancy occurs, the gastrointestinal tract is capable of increasing the absorption of iron to about 20% to 30% of the increasing the absorption of iron to about 20% to 30% of the total daily intake.total daily intake.

When iron deficiency anemia is severe, iron preparations can When iron deficiency anemia is severe, iron preparations can be administered intramuscularly. Such preparations are be administered intramuscularly. Such preparations are administered using the Z-track best practice methodadministered using the Z-track best practice method

Common food sources of iron, vitamin b12, and folic acid


Vitamin B12 Deficiency Anemia

OverviewOverview Proper production of RBCs depends on adequate Proper production of RBCs depends on adequate

desoxyribonucleic acid (DNA) synthesis in the precursor cells so desoxyribonucleic acid (DNA) synthesis in the precursor cells so that cell division and maturation into functional RBCs can occur. that cell division and maturation into functional RBCs can occur.

All DNA synthesis requires adequate amounts of folik acid to All DNA synthesis requires adequate amounts of folik acid to ensure the availability of the nucleotide thymidine,ensure the availability of the nucleotide thymidine, which which stimulates DNA synthesis. One function of vitamin Bstimulates DNA synthesis. One function of vitamin B1212 is to serve is to serve as a cofactor to activate the enzyme system responsible for as a cofactor to activate the enzyme system responsible for transporting folic acid into the cell, where DNA synthesis occurs. transporting folic acid into the cell, where DNA synthesis occurs.

Thus a deficiency of vitamin BThus a deficiency of vitamin B1212 indirectly causes anemia by indirectly causes anemia by inhibiting folic acid transportation and limiting DNA synthesis in inhibiting folic acid transportation and limiting DNA synthesis in RBC precursor cells. RBC precursor cells.

These precursor cells then undergo improper DNA synthesis and These precursor cells then undergo improper DNA synthesis and increase in size. Only a few are released from the bone marrow. increase in size. Only a few are released from the bone marrow. This type of anemia is called This type of anemia is called megaloblastic (macrocytic) megaloblastic (macrocytic) because of the large size of these abnormal cells.because of the large size of these abnormal cells.



Vitamin BVitamin B1212 deficiency can result from inadequate intake (dietary deficiency can result from inadequate intake (dietary deficiency). This can occur with strict vegetarian diets or diets deficiency). This can occur with strict vegetarian diets or diets lacking sufficient dairy products. lacking sufficient dairy products.

Conditions such as small bowel resection, diverticula, tapeworm, Conditions such as small bowel resection, diverticula, tapeworm, or overgrowth of intestinal bacteria can lead to poor absorption of or overgrowth of intestinal bacteria can lead to poor absorption of vitamin Bvitamin B1212 from the intestinal tract. from the intestinal tract.

Anemia caused by failure to absorb vitamin BAnemia caused by failure to absorb vitamin B1212 (pernicious (pernicious anemia) anemia) can also result from a deficiency of can also result from a deficiency of intrinsic factor intrinsic factor (a (a substance normally secreted by the gastric mucosa), which is substance normally secreted by the gastric mucosa), which is necessary for intestinal absorption of vitamin Bnecessary for intestinal absorption of vitamin B1212..

Vitamin BVitamin B1212 deficiency anemia may be mild or severe, usually deficiency anemia may be mild or severe, usually develops slowly, and produces few symptoms. Clients usually develops slowly, and produces few symptoms. Clients usually have pallor and jaundice, as well as have pallor and jaundice, as well as glossitis glossitis (a smooth, beefy-(a smooth, beefy-red tongue), fatigue, and weight loss. red tongue), fatigue, and weight loss.

Because vitamin BBecause vitamin B1212 also is necessary for normal nervous system also is necessary for normal nervous system functioning, especially of the peripheral nerves, clients with functioning, especially of the peripheral nerves, clients with pernicious anemia may also have neurologic abnormalities, such pernicious anemia may also have neurologic abnormalities, such as as paresthesias paresthesias (abnormal sensations) in the feet and hands and (abnormal sensations) in the feet and hands and disturbances of balance and gait.disturbances of balance and gait.



Collaborative managementCollaborative management When anemia is caused by a When anemia is caused by a

dietary deficiency, the client must dietary deficiency, the client must increase the intake of foods rich increase the intake of foods rich in vitamin B12 (animal proteins, in vitamin B12 (animal proteins, eggs, dairy products). eggs, dairy products).

Vitamin supplements may be Vitamin supplements may be prescribed when anemia is prescribed when anemia is severe. For clients who have severe. For clients who have anemia asanemia as a result of a deficiency a result of a deficiency of intrinsic factor, vitamin B12 of intrinsic factor, vitamin B12 must be administered must be administered parenterally on a regular parenterally on a regular schedule (usually weekly for schedule (usually weekly for initial treatment, then monthly for initial treatment, then monthly for maintenance).maintenance).


Folic Acid Deficiency Anemia

OverviewOverview Primary folic acid deficiency can also cause megaloblastic anemia. Primary folic acid deficiency can also cause megaloblastic anemia.

Clinical manifestations are similar to those of vitamin BClinical manifestations are similar to those of vitamin B1212 deficiency deficiency without the accompanying nervous system manifestations, because without the accompanying nervous system manifestations, because folic acid does not appear to affect nerve function. folic acid does not appear to affect nerve function.

The absence of neurologic problems is an important diagnostic finding The absence of neurologic problems is an important diagnostic finding to differentiate folic acid deficiency from vitamin Bto differentiate folic acid deficiency from vitamin B1212 deficiency. The deficiency. The disease develops slowly, and symptoms may be attributed to other disease develops slowly, and symptoms may be attributed to other problems or diseases.problems or diseases.

The three common causes of folic acid deficiency are poor nutrition, The three common causes of folic acid deficiency are poor nutrition, malabsorption, and drugs. Poor nutrition, especially a diet lacking malabsorption, and drugs. Poor nutrition, especially a diet lacking green leafy vegetables, liver, yeast, citrus fruits, dried beans, and green leafy vegetables, liver, yeast, citrus fruits, dried beans, and nuts, is the most common cause. Chronic alcohol abuse and parenteral nuts, is the most common cause. Chronic alcohol abuse and parenteral alimentation without folic acid supplementation are other dietary alimentation without folic acid supplementation are other dietary causes. causes.

Malabsorption syndromes, such as Crohn's disease, are the second Malabsorption syndromes, such as Crohn's disease, are the second most common cause.most common cause.

Specific drugs impede the absorption and conversion of folic acid to its Specific drugs impede the absorption and conversion of folic acid to its active form and can also lead to folic acid deficiency and anemia. Such active form and can also lead to folic acid deficiency and anemia. Such drugs include methotrexate, some anticonvulsants, and oral drugs include methotrexate, some anticonvulsants, and oral contraceptivescontraceptives


Folic Acid Deficiency Anemia

Collaborative managementCollaborative management Prevention of folic acid deficiency anemia is aimed at Prevention of folic acid deficiency anemia is aimed at

identifying high-risk clients, such as older, debilitated clients identifying high-risk clients, such as older, debilitated clients with alcoholism; clients prone to malnutrition; and those with with alcoholism; clients prone to malnutrition; and those with increased folic acid requirements. increased folic acid requirements.

A diet high in folic acid and vitamin BA diet high in folic acid and vitamin B1212 prevents a deficiency. prevents a deficiency.

By routinely including assessment of dietary habits in a By routinely including assessment of dietary habits in a health history, the nurse can determine which clients are at health history, the nurse can determine which clients are at risk for diet-induced anemias and provide appropriate follow-risk for diet-induced anemias and provide appropriate follow-up. up.

For the client diagnosed with this type of anemia, For the client diagnosed with this type of anemia, management includes oral folic acid 1 mg daily or management includes oral folic acid 1 mg daily or intramuscular administration of folic acid for clients with intramuscular administration of folic acid for clients with absorption problems.absorption problems.


Aplastic Anemia

OverviewOverview Aplastic anemia Aplastic anemia is a deficiency of circulating erythrocytes is a deficiency of circulating erythrocytes

resulting from arrested development of RBCs within the bone resulting from arrested development of RBCs within the bone marrow. It is caused by an injury to the hematopoietic precursor marrow. It is caused by an injury to the hematopoietic precursor cell, the cell, the pluripotent stem cell. pluripotent stem cell.

Although aplastic anemiaAlthough aplastic anemia sometimes occurs alone, it is usually sometimes occurs alone, it is usually accompanied by accompanied by agranulocytopenia agranulocytopenia (a reduction in leukocytes) (a reduction in leukocytes) and and thrombocytopenia thrombocytopenia (a reduction in platelets). (a reduction in platelets).

These three problems occur at the same time because the bone These three problems occur at the same time because the bone marrow produces not only RBCs but also white blood cells (WBCs) marrow produces not only RBCs but also white blood cells (WBCs) and platelets.and platelets.

Consequently, if the bone marrow is abnormal for any reason or if Consequently, if the bone marrow is abnormal for any reason or if it has been exposed to a toxic substance that can damage bone it has been exposed to a toxic substance that can damage bone marrow cells, production of erythrocytes, leukocytes, and marrow cells, production of erythrocytes, leukocytes, and thrombocytes slows greatly. thrombocytes slows greatly. Pancytopenia Pancytopenia (a deficiency of all (a deficiency of all three cell types) is common in aplastic anemia. three cell types) is common in aplastic anemia.


Aplastic Anemia

The onset of aplastic anemia may be The onset of aplastic anemia may be insidious or rapid.insidious or rapid.

The development of aplastic anemia, The development of aplastic anemia, although relatively rare, is associated with although relatively rare, is associated with chronic exposure to several toxic agents. chronic exposure to several toxic agents. In about 50% of cases, the cause of In about 50% of cases, the cause of aplastic anemia is unknown. Aplastic aplastic anemia is unknown. Aplastic anemia may occur as an aftermath of viral anemia may occur as an aftermath of viral infection, but the mechanism of bone infection, but the mechanism of bone marrow damage is unknownmarrow damage is unknown


Aplastic Anemia

Collaborative managementCollaborative management Blood transfusions are the mainstay of treatment for clients Blood transfusions are the mainstay of treatment for clients

with aplastic anemia.with aplastic anemia.

Transfusion is indicated only when the anemia causes real Transfusion is indicated only when the anemia causes real disability or when bleeding is life threatening because of disability or when bleeding is life threatening because of thrombocytopenia. thrombocytopenia.

Unnecessary transfusion, however, increases the Unnecessary transfusion, however, increases the opportunity for the development of immune reactions to opportunity for the development of immune reactions to platelets, shortens the life span of the transfused cell, and platelets, shortens the life span of the transfused cell, and may increase the rate of rejection of trans planted marrow may increase the rate of rejection of trans planted marrow cells. Thus transfusions are discontinued as soon as the cells. Thus transfusions are discontinued as soon as the bone marrow begins to produce RBCs.bone marrow begins to produce RBCs.


Aplastic Anemia

Because clients with some types of aplastic anemia have a Because clients with some types of aplastic anemia have a disease course similar to that of autoimmune problems, disease course similar to that of autoimmune problems, immunosuppressive therapy may be helpful. Agents that immunosuppressive therapy may be helpful. Agents that selectively suppress lymphocyte activity, such as selectively suppress lymphocyte activity, such as antilymphocyte globulin (ALG), antithymocyte globulin (ATG), antilymphocyte globulin (ALG), antithymocyte globulin (ATG), and cyclosporine (Sandimmune), have brought about partial or and cyclosporine (Sandimmune), have brought about partial or complete remissions. In more severe cases, general complete remissions. In more severe cases, general immunosuppressive agents, such as prednisone and immunosuppressive agents, such as prednisone and cyclophosphamide (Cytoxan, Procytox), have been effective.cyclophosphamide (Cytoxan, Procytox), have been effective.

Splenectomy Splenectomy (removal of the spleen) is considered in clients (removal of the spleen) is considered in clients with an enlarged spleen that is either destroying normal RBCs with an enlarged spleen that is either destroying normal RBCs or suppressing their development. or suppressing their development.

Bone marrow trans plantation, which replaces defective stem Bone marrow trans plantation, which replaces defective stem cells, has also resulted in a cure for some clients. Cost, cells, has also resulted in a cure for some clients. Cost, availability, and complications limit this technique for availability, and complications limit this technique for treatment of aplastic anemia, howevertreatment of aplastic anemia, however

POLYCYTHEMIAPOLYCYTHEMIA In polycythemia, the number of red blood In polycythemia, the number of red blood

cells (RBCs) in whole blood is greater than cells (RBCs) in whole blood is greater than normal. normal.

The blood of a client with polycythemia is The blood of a client with polycythemia is hyperviscous hyperviscous (thicker than normal blood). (thicker than normal blood).

The problem may be temporary (occurring The problem may be temporary (occurring as a result of other conditions) or chronic. as a result of other conditions) or chronic. One type of polycythemia, polycythemia One type of polycythemia, polycythemia vera, is fatal if left untreated.vera, is fatal if left untreated.

Polycythemia VeraPolycythemia Vera OverviewOverview Polycythemia vera (PV) Polycythemia vera (PV) is characterized by a sustained is characterized by a sustained

increase in blood hemoglobin concentration to 18 g/dL, an increase in blood hemoglobin concentration to 18 g/dL, an RBC count of 6 million/mm3, or a hematocrit increase to 55% RBC count of 6 million/mm3, or a hematocrit increase to 55% or greater. or greater.

PV is a cancer of the RBCs with three major hallPV is a cancer of the RBCs with three major hall marks: marks: continuous production of massive numbers of RBCs, continuous production of massive numbers of RBCs, excessive leukocyte production, and overproduction of excessive leukocyte production, and overproduction of thrombocytes. Extreme thrombocytes. Extreme hypercellularity hypercellularity (cell excess) of the (cell excess) of the peripheral blood occurs in people with PVperipheral blood occurs in people with PV

The skin, especially facial, and mucous membranes have a The skin, especially facial, and mucous membranes have a dark, flushed dark, flushed (plethoric) (plethoric) appearance. These areas may appearance. These areas may appear purplish or cyanotic because the blood in these appear purplish or cyanotic because the blood in these tissues is incompletely oxygenated. Most clients experience tissues is incompletely oxygenated. Most clients experience intense itching related to vasodilation and variation in tissue intense itching related to vasodilation and variation in tissue oxygenation. oxygenation.

Polycythemia VeraPolycythemia Vera Blood viscosity is also greatly increased, causing a Blood viscosity is also greatly increased, causing a

corresponding increase in peripheral resistance. corresponding increase in peripheral resistance.

Superficial veins are visibly distended. Blood moves more Superficial veins are visibly distended. Blood moves more slowly through all tissues and thus places increased slowly through all tissues and thus places increased demands on the pumping action of the heart, resulting in demands on the pumping action of the heart, resulting in hypertension. In some highly vascular areas, blood flow hypertension. In some highly vascular areas, blood flow may become so slow that vascular stasis occurs. Vascular may become so slow that vascular stasis occurs. Vascular stasis causes stasis causes thrombosis thrombosis (clot formation) within the (clot formation) within the smaller vessels to the extent that the vessels are occluded smaller vessels to the extent that the vessels are occluded and the surrounding tissues experience hypoxia, and the surrounding tissues experience hypoxia, progressing to anoxia and further to infarction and necrosis. progressing to anoxia and further to infarction and necrosis. Tissues most prone to this complication are the heart, Tissues most prone to this complication are the heart, spleen, and kidneys, although infarction with loss of tissue spleen, and kidneys, although infarction with loss of tissue and organ function can occur in any organ or tissueand organ function can occur in any organ or tissue

Polycythemia VeraPolycythemia Vera Because the actual number of cells in the blood is greatly Because the actual number of cells in the blood is greatly

increased and the cells are not completely normal, increased and the cells are not completely normal, individual cell life spans are shorter. The shorter life spans, individual cell life spans are shorter. The shorter life spans, coupled with increased cell production, result in a rapid coupled with increased cell production, result in a rapid turnover of peripheral blood cells. This rapid turnover turnover of peripheral blood cells. This rapid turnover increases the amount of intracellular products (released increases the amount of intracellular products (released when cells die) in the blood, adding to the general when cells die) in the blood, adding to the general "sludging" of the blood. These products include uric acid "sludging" of the blood. These products include uric acid and potassium, which cause the symptoms of gout and and potassium, which cause the symptoms of gout and hyperkalemia hyperkalemia (elevated serum potassium level).(elevated serum potassium level).

Later clinical manifestations of PV are related to abnormal Later clinical manifestations of PV are related to abnormal blood cells. Even though the number of circulating blood cells. Even though the number of circulating erythrocytes is greatly increased, their oxygencarrying erythrocytes is greatly increased, their oxygencarrying capacity is impaired, and clients experience severe capacity is impaired, and clients experience severe generalized hypoxia. In spite of the RBC excess, most generalized hypoxia. In spite of the RBC excess, most clients with PV are susceptible to bleeding problems clients with PV are susceptible to bleeding problems because of an associated platelet dysfunctionbecause of an associated platelet dysfunction

Polycythemia VeraPolycythemia VeraCollaborative managementCollaborative management Polycythemia vera is a malignant disease that progresses in severity Polycythemia vera is a malignant disease that progresses in severity

over time. If left untreated, few people with PV liveover time. If left untreated, few people with PV live longer than 2 longer than 2 years. years.

Conservative management with repeated phlebotomies (two to five Conservative management with repeated phlebotomies (two to five times per week) can prolong life for 5 to 10 years. times per week) can prolong life for 5 to 10 years. (Phlebotomy (Phlebotomy is is the collection of the client's RBCs to decrease the number of RBCs the collection of the client's RBCs to decrease the number of RBCs and diminish blood viscosity.) and diminish blood viscosity.)

Maintaining adequate hydration and promoting venous return are Maintaining adequate hydration and promoting venous return are essential to prevent thrombus formation. Therapy aims to prevent essential to prevent thrombus formation. Therapy aims to prevent clot formation and includes the use of anticoagulantsclot formation and includes the use of anticoagulants

As the disease progresses, clients need more intensive therapies As the disease progresses, clients need more intensive therapies that suppress bone marrow activity, including oral alkylating agents that suppress bone marrow activity, including oral alkylating agents and/or irradiation with injections of radioactive phosphorus. and/or irradiation with injections of radioactive phosphorus.

Bone marrow transplantation, an experimental treatment, is Bone marrow transplantation, an experimental treatment, is promising, but the results are too limited to determine its application promising, but the results are too limited to determine its application to PVto PV

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Interventions for Clients with Hematologic Problems RED BLOOD CELL DISORDERS