Hematologic Disorders

CHAPTER 8 Hematologic Disorders

Section One Disorders of the Red Blood Cells

Anemia is a common hematopoietic disorder defined as reduced RBC volume (hematocrit [Hct]) or reduced concentration of hemoglobin (Hgb). Consideration of the patient’s intravascular volume (i.e., hydration status) is essential for proper interpretation of Hct and Hgb values. The general effects of anemia are the result of a deficiency in the O₂-carrying mechanism, although some effects are related to varied pathogenetic factors.

Anemias can be classified in two ways: (1) those involving diminished production or accelerated loss of RBCs (etiology) or (2) those involving cell size (morphology). For details, see TABLES 8-1 and 8-2. Three common types of anemias are discussed in this section: anemia of chronic disease, hemolytic and sickle cell anemia, and hypoplastic (aplastic) anemia.

TABLE 8-1 ETIOLOGIC (PATHOPHYSIOLOGIC) CLASSIFICATIONS OF ANEMIA

TYPE	CAUSE	DISEASES
Decreased or defective production of erythrocytes	Altered hemoglobin synthesis	Iron deficiency
		Thalassemia
		Anemia of chronic inflammation or disease
	Altered DNA synthesis from deficient nutrients	Pernicious anemia (decreased vitamin B₁₂, folate)
	Stem cell dysfunction	Aplastic anemia, myeloproliferative leukemia or dysplasia
	Bone marrow infiltration	Carcinoma, lymphoma, multiple myeloma
	Autoimmune disease or idiopathic	Pure red cell aplasia
Increased erythrocyte destruction	Blood loss	Acute (hemorrhage, trauma)
		Chronic (gastrointestinal bleeding, menorrhagia)
	Hemolysis (intrinsic)	Hereditary spherocytosis, sickle cell trait or disease, pyruvate kinase deficiency, glucose-6-phosphate dehydrogenase (G6PD) deficiency
	Hemolysis (extrinsic)	Warm or cold antibody disease, infection (malarial, clostridial), erythrocyte trauma (hemolytic uremic syndrome, TTP, mechanical cardiac valve, paravalvular leak), splenic sequestration, burns

TTP, Thrombotic thrombocytopenic purpura.

TABLE 8-2 MORPHOLOGIC CLASSIFICATIONS OF ANEMIA

Anemia of Chronic Disease

Overview/Pathophysiology

Erythropoietin (EPO) is a naturally occurring protein hormone produced and released by the kidneys (90%) and liver (10%). The kidneys are stimulated to release EPO in response to low blood oxygenation. EPO then stimulates stem cells in the bone marrow to develop and produce RBCs. Individuals with decreased renal function (e.g., chronic kidney disease [CKD]) often become anemic because their kidneys cannot produce EPO. In other chronic conditions, bone marrow fails to compensate for decreased RBC survival adequately by increasing RBC production. In these cases, EPO rarely is an important cause of underproduction of RBCs except in renal failure. However, the development of recombinant human EPO (epoetin alfa) has provided dramatic benefits for patients with CKD, patients receiving chemotherapy for cancer, and patients undergoing treatment for human immunodeficiency virus (HIV) infection.

Nursing Diagnoses and Interventions

Activity intolerance

related to anemia

Desired outcome

After treatment, Hgb and Hct levels are within medical goals, and patient perceives exertion at 3 or less on a 0-10 scale and tolerates activity, as evidenced by respiratory rate (RR) 12-20 breaths/min, presence of eupnea, heart rate (HR) 100 beats per minute (bpm) or less, and absence of dizziness and headaches.

Nursing Interventions

♦ Assess patient for dyspnea on exertion, dizziness, palpations, headaches, and verbalization of increased exertion level. Ask patient to rate perceived exertion, to correctly interpret patient’s breathing difficulty.

♦ Monitor oximetry as indicated. Report O₂ saturation 92% or less. Administer oxygen as prescribed, and encourage deep breathing to augment oxygen delivery to the tissues.

♦ Assess patient for ambulatory ability and implement appropriate strategies to minimize risk for falls and/or injury.

♦ Facilitate coordination of care allowing time for at least 90 min of rest as needed between care activities, to provide periods of undisturbed rest.

♦ Encourage gradually increasing activities to patient’s tolerance. Set mutually agreed on goals with patient (e.g., “Let’s plan this morning’s activity goals. Do you think you could walk up and down the hall once, or twice?” or appropriate amount, depending on patient’s tolerance). Allows time for patient’s condition to improve.

♦ Reassure patient that symptoms usually are relieved and tolerance for activity increases with therapy. May reduce anxiety, which depletes energy.

♦ Administer blood components (usually packed RBCs) as prescribed. Double-check type and crossmatching and patient identifiers with a colleague, and monitor for and report signs of transfusion reaction. Replenishes needed RBCs.

Patient-Family Teaching and Discharge Planning

Include verbal and written information about the following:

♦ Importance of a well-balanced diet, especially iron intake, if appropriate, which is found in foods such as red meat, dark green vegetables, legumes, and certain fruits (apricots, figs, raisins). Refer to clinical dietitian as prescribed.

♦ Special instructions for taking iron, if appropriate, depending on type prescribed. Therapy may need to be continued for 4-6 mo to replace iron stores adequately.

♦ Necessity for EPO replacement therapy to be continued for duration of the underlying condition.

♦ When self-administering EPO, importance of not shaking medication vial before taking it. Shaking the vial may denature glycoprotein in the solution and render it biologically inactive. Any discolored solution or solution with particulate matter should not be used.

♦ Other medications, including drug names, dosage, purpose, schedule, precautions, and potential side effects. Also discuss drug/drug, herb/drug, and food/drug interactions.

♦ Risks and benefits of RBC transfusion (as explained by health care provider) and necessity for signed consent for the transfusion.

Hemolytic and Sickle Cell Anemia

Overview/Pathophysiology

Hemolytic anemia is characterized by abnormal or premature destruction of RBCs. Hemolysis can occur because of intrinsic or extrinsic factors (Table 8-1), for example, from a foreign antigen (e.g., from a transfusion reaction) or an autoimmune reaction in which the hemolytic agent is intrinsic to the patient’s body. Other possible causes include exposure to radiation and ingestion of certain medications (e.g., sulfisoxazole, phenytoin, methyldopa). Acquired hemolytic anemia is usually the result of an abnormal immune response that causes premature destruction of RBCs.

Hemolytic crisis

Individuals with chronic hemolytic anemia may do relatively well for a time, but many factors can precipitate a hemolytic crisis or acute hemolysis (i.e., an individual with mild hemolytic anemia can become severely anemic with an acute infectious process or with any other physiologic or emotional stressor, including surgery, trauma, or emotional upset). Widespread hemolysis causes an acute decrease in O₂-carrying capacity of the blood that results in decreased O₂ delivery to the tissues. Organ congestion from the hemolyzed blood cells occurs, and it precipitates organ dysfunction and a shock state. Although medical therapy has improved the general prognosis of these patients, there is still an overall decrease in life expectancy.

Sickle cell anemia is a genetic disorder of hemoglobin (Hgb) synthesis characterized by the presence of 50% or more sickle Hgb (HbS). It results in abnormal, crescent-shaped, rigid, and elongated erythrocytes. Because of their abnormal shape and rigidity, these “sickled” RBCs interfere with circulation because they are not pliable like normal cells. Because they cannot get through the microcirculation smoothly, they are destroyed in the process (hemolysis). Vasoocclusive phenomena and hemolysis are the clinical hallmarks of sickle cell disease. Sickle cell anemia can affect almost every body system through decreased O₂ delivery, decreased circulation caused by occlusion of the vessels by RBCs, and the inflammatory process leading to infection. Sickle cell disease occurs when the gene is inherited from both parents (homozygous). A carrier state for sickle cell trait exists when it is inherited from only one parent (heterozygous).

Acute chest syndrome (ACS)

ACS is the leading cause of death in adolescents and adults with sickle cell disease. Infection is believed to be the etiologic disorder in most cases, but ACS also can occur because of thrombosis, infarction, fat embolism, and atelectasis.

Assessment

Signs and symptoms/physical findings

Chronic indicators

Pallor (e.g., conjunctival), fatigue, dyspnea on exertion, and intermittent dizziness, all of which depend on anemia severity; distorted skeletal growth, and increased potential for hematogenic osteomyelitis in children and adolescents; and jaundice, arthritis, cholelithiasis, retinopathy, renal failure, skin ulcers, and chronic organ damage (heart, liver, lungs) from chronic hemolytic anemia.

Acute indicators (hemolytic crisis)

Fever; headache; visual blurring or temporary blindness; severe abdominal pain; vomiting; splenomegaly; hepatomegaly; back, lower leg, and joint pain; priapism; palpitations; shortness of breath (because of pulmonary sequestration, anemia, infection); aplastic crisis (resulting from transient marrow suppression by viruses); chills; lymphadenopathy; decreased urinary output; and stroke. Peripheral nerve damage can result in paralysis or paresthesias. Occasionally a low-grade fever may occur 1-2 days after a crisis event. Attacks last a few hours to a few days and resolve spontaneously.

ACS

Chest pain, fever, cough.

History and risk factors

Family history, African-American race, and less commonly, hereditary descendants of Greece and southern Italy.

Diagnostic Tests

Sickle cell test (Sickledex)

Screens for sickle cell anemia or its trait.

Hgb electrophoresis

Discriminates among Hgb AS, sickle cell trait, and Hgb SS, sickle cell anemia.

Cold agglutinin titer

Markedly elevated (greater than 1 : 1000). This test measures antibodies that are able to clump RBCs at cold temperatures.

Hgb and Hct

Decreased because of RBC destruction.

Urine and fecal urobilinogen

Levels increased. These are more sensitive indicators of RBC destruction than serum bilirubin levels.

Bone marrow aspiration

Reveals erythroid hyperplasia, especially with chronic hemolytic anemia.

Reticulocyte count

Elevated because of the rapid destruction of RBCs.

Serum haptoglobin

Decreased because the Hgb released from hemolyzed RBCs is bound to haptoglobin.

Chorionic biopsy

Determines presence of sickle cell disease in the first 6-8 wk of pregnancy.

Collaborative Management

The goals of treatment for sickle cell anemia are relieving symptoms and preventing crisis and complications.

Elimination or discontinuation of causative factor

If possible (e.g., chemical, drug, incompatible blood).

Volume replacement

Adequate hydration is important to prevent complications from decreased organ perfusion secondary to hemolysis or sickle cell vascular congestion.

O₂ therapy

Routine pulse oximetry determines need for oxygen therapy for patients who are hypoxemic (e.g., O₂ saturation 92% or less).

Supportive therapy for shock state

For example, antibiotics, vasopressors.

Transfusion

For circulatory failure or severe anemic anoxia if it occurs. Especially at risk are older persons and those with limited cardiopulmonary reserves. A conservative approach is taken and the patient’s Hgb is usually not raised greater than 10 g/dL because of increased viscosity with higher levels, development of alloantibodies, and iron overload.

Iron chelation

Agents such as deferoxamine mesylate may be used to bind iron so that chronically transfused patients (i.e., those with sickle cell disease or thalassemia) have a reduced risk of iron overload, which can lead to end-organ damage to the liver and heart.

Erythrocytapheresis (RBC exchange or partial exchange)

A procedure that removes abnormal RBCs and infuses healthy RBCs with or without normal saline to correct the anemia. It is used for younger patients with a history of stroke or for individuals with pulmonary or cardiac disease.

Corticosteroids

Help stabilize cell membranes and decrease the inflammatory response.

Folic acid

Helps prevent hemolytic crisis by increasing RBC production in individuals with chronic hemolytic anemias.

Hydroxyurea

Decreases painful sickling episodes by stimulating the production of fetal Hgb.

Rituximab

Monoclonal antibody given by the IV route weekly for 4 wk as an antibody suppressant.

Danazol

Androgen with unknown mechanism of action.

IV immune globulin

Causes binding of antibodies so that they are less likely to cause hemolysis.

Immunosuppressive agents (e.g., cyclophosphamide, azathioprine, or cyclosporine)

Suppress the antibodies causing RBC lysis.

Influenza and pneumococcal vaccinations

Prevent infection that may trigger crisis.

Splenectomy

Provides symptomatic relief, depending on anemia cause. It also may be done prophylactically to reduce the potential for rupture and massive blood loss. The spleen is the site of RBC destruction.

Stem cell transplant from human leukocyte antigen (HLA)–matched donor following high-dose chemotherapy

Can be curative in young patients with sickle cell disease. This treatment potentially ablates the genetic disorder and restores normal hematopoiesis. However, it can have acute and delayed risks of morbidity and mortality.

Pain management

Aggressive use of opioids and other analgesics in acute crisis; meperidine is contraindicated because of increased side effects, including seizures, in high doses.

Nursing Diagnoses and Interventions

Acute pain

Chronic pain

related to joint hemolysis secondary to hemolytic crisis or sickle cell disease

Desired outcomes

Within 1 hr of intervention, patient’s subjective perception of discomfort decreases, as documented by pain scale. Objective indicators, such as grimacing, are absent or diminished. Lifestyle behaviors are not compromised because of discomfort.

Nursing Interventions

♦ Monitor for pain by having patient rate discomfort on a scale of 0 (no pain) to 10 (worst pain) to determine patient’s perception of pain/discomfort.

♦ Instruct patient to request analgesic before pain becomes too intense. Pain is easier to control if treated before it becomes severe.

♦ Administer analgesia as prescribed to relieve and/or control pain.

♦ Reassure patient that acute pain will subside when acute hemolytic episode is over. If chronic pain persists, ensure that a plan for pain management upon discharge is addressed by health care provider.

♦ Assist with positioning as needed to promote comfort.

♦ Apply moist heat packs to painful joints, but use cautiously for patients with decreased peripheral sensations. Increases circulation and decreases pain.

♦ Apply elastic stockings or wraps, if prescribed, to protect skin, support joints, and promote circulation.

Ineffective peripheral and cardiopulmonary tissue perfusion

related to inflammatory process and occlusion of blood vessels with RBCs

Desired outcome

Following treatment, patient has adequate peripheral and cardiopulmonary perfusion, as evidenced by SBP 10 mm Hg or less lower than baseline SBP, peripheral pulses 2+ or more on a 0-4+ scale, heart rate (HR) 100 beats per minute (bpm) or less, respiratory rate (RR) 12-20 breaths/min with normal depth and pattern (eupnea), and normal skin color.

Nursing Interventions

♦ Assess SBP at frequent intervals, and report significant drops (more than 10 mm Hg from baseline readings).

♦ Assess peripheral pulses. Be alert to pulses 2+ or less on a 0-4+ scale. Indicates degree of peripheral perfusion.

♦ Monitor for decreased BP, increased HR, decreased pulse amplitude, dyspnea, and decreased urine output. Indicate cardiac depression.

♦ Assess for and report increased RR, dyspnea, shortness of breath, cyanosis, and pallor; pulse oximetry; O₂ saturation 92%, or less, which could signal ACS.

♦ Monitor lips, tongue, oral mucosa, and nail beds. These areas are especially important to monitor in dark-skinned individuals because pallor is difficult to assess. Indicates hypoxia or respiratory dysfunction.

♦ Administer oxygen as prescribed, to counter hypoxemia if present.

♦ Assist with ROM exercises and positioning. Be aware that exercise should be avoided if any early signs of hemolytic crisis appear because exercise can aggravate hemolysis. In the absence of crisis, exercise enhances tissue perfusion and maintains and increases joint mobility.

♦ Report significant findings to patient’s health care provider.

Risk for impaired skin integrity

Impaired tissue integrity

related to altered circulation (occlusion of the vessels), resulting in impaired oxygen transport to tissues and skin

Desired outcome

Patient’s skin and tissue remain nonerythematous and intact.

Patient-Family Teaching and Discharge Planning

Include verbal and written information about the following:

♦ Rationale of treatment plan and importance of medical follow-up.

♦ Importance of ensuring that fluid intake is adequate to promote organ and tissue perfusion and avoiding high altitudes for individuals who are hypoxemic.

♦ Phone numbers and contact person for local and/or national support groups available for sickle cell anemia, such as:

Sickle Cell Disease Association of America: http://SickleCellDisease.org

♦ Indicators of hemolytic crisis, including jaundice, dyspnea, shortness of breath, joint or abdominal pain, decreasing BP, and increased HR; and factors that precipitate hemolytic crisis, such as emotional stress, physical stress, infection, trauma, chemicals, and toxic drug reactions (e.g., to penicillin, methyldopa, sulfonamides, quinine).

♦ Importance of maintaining a calm environment. Teach stress reduction techniques, such as meditation and relaxation exercises.

♦ Importance of hygiene and avoiding infectious processes, such as upper respiratory infections, and getting appropriate immunizations and prompt medical attention if infection occurs.

♦ Necessity to avoid intake of iron and citrus with meals (citrus increases absorption of iron) to help minimize abnormal RBC proliferation.

♦ Medications, including drug names, purpose, schedule, dosage, precautions, and potential side effects. Discuss drug/drug, herb/drug, and food/drug interactions. Over-the-counter (OTC) drugs must be avoided unless approved by primary health care provider, along with alcohol and smoking. Corticosteriods increase the risk for gastrointestinal (GI) hemorrhage, delayed wound healing, and increased appetite. Review need to take medication with food, immediately take missed doses, and avoid precipitously discontinuing medication.

♦ Risks and benefits of RBC transfusion (as explained by health care provider) and necessity for patient to sign consent for the transfusion.

♦ Birth control options and referrals for genetic counseling and any needed psychosocial support for living with a chronic illness.

♦ Importance of obtaining a MedicAlert bracelet and identification card outlining diagnosis and emergency treatment:

MedicAlert Foundation: www.medicalert.org

Aplastic Anemia

Overview/Pathophysiology

Aplastic anemia results from inability of erythrocyte-producing organs, specifically bone marrow stem cells, to produce erythrocytes. Causes of aplastic anemia are varied but can include use of antineoplastic or antimicrobial agents, infectious process, systemic lupus erythematosus (SLE), pregnancy, hepatitis, and radiation. Approximately half of patients with aplastic anemia have had exposure to drugs or chemical agents (benzene, insecticides), whereas the remaining half have had immunologic disorders. For no identified etiology, the cause is believed to be a T-cell–mediated autoimmune disorder attacking the patient’s own bone marrow. Aplastic anemia most often involves pancytopenia—depressed production of all three bone marrow elements: erythrocytes, platelets, and granulocytes (especially neutrophils) without abnormal cellular morphologies. Usually the onset is insidious, but it can evolve quickly in some cases. Prognosis usually is poor.

Tags: Manual of Medical-Surgical Nursing Care Nursing Interventions an

Sep 1, 2016 | Posted by admin in NURSING | Comments Off

Full access? Get Clinical Tree

Get Clinical Tree app for offline access

Get Clinical Tree app for offline access