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1. Hematology Disorders BY ISHAQ ARSHAD

2. Sickle cell anemia Immuno hemolytic Anaemia Iron deficiency anemia Vitamin B12 & Folic acid deficiency anemia Aplastic anemia Polycythemia Contents:

3. Sickle cell anemia

4. Sickle cell anemia is a severe hemolytic anemia that results from inheritance of the sickle hemoglobin gene. This gene causes the hemoglobin molecule to be defective. The sickle hemoglobin (HbS) acquires a crystal-like formation when exposed to low oxygen tension. The oxygen level in venous blood can be low enough to cause this change; Consequently, the RBC containing (HbS) loses its round, very pliable, biconcave disk shape and becomes deformed, rigid, and sickle-shaped .

5. These long, rigid RBCs can adhere to the endothelium of small vessels; when they pile up against each other, blood flow to a region or an organ may be reduced (Hoffman, et al., 2000). If ischemia or infarction results, the patient may have pain, swelling, and fever.

6. There are three types of sickle cell crisis in the adult population. The most common is the very painful sickle crisis , which results from tissue hypoxia and necrosis due to inadequate blood flow to a specific region of tissue or organ. Aplastic crisis results from infection with the human parvovirus. The hemoglobin level falls rapidly and the marrow cannot compensate, as evidenced by an absence of reticulocytes. Sequestration crisis results when other organs pool the sickled cells. Although the spleen is the most common organ responsible for sequestration in children, by 10 years of age most children with sickle cell anemia have had a splenic infarction and the spleen is then no longer functional (autosplenectomy). In adults, the common organs involved in sequestration are the liver and, more seriously, the lungs. SICKLE CELL CRISIS

7. Acute chest syndrome is manifested by a rapidly falling hemoglobin level, tachycardia, fever , and bilateral infiltrates seen on the chest x-ray . These signs often mimic infection; in fact, recent studies have identified infection as a major cause of acute chest syndrome ( Vichinsky , et al., 2000). Another common cause is pulmonary fat embolism. ACUTE CHEST SYNDROME

8. • Acute pain (especially back, chest, and long bones) from vascular occlusion of the small vessels as the sickled cells clump • Fever as body responds to acute sickling episode and accompanying provoking event • Painful, swollen joints due to vaso-occlusive process • Fatigue due to chronic anemia • Stroke (cerebrovascular accident) due to vaso-occlusive process • Enlarged liver ( hepatomegaly ) • Enlarged heart and systolic murmur Clinical Manifestations

9. • Low RBC count due to chronic hemolytic anemia; the RBCs have a shorter lifespan. • Elevated WBCs. • Increased reticulocytes. • Presence of Howell-Jolly bodies and target cells. • Sickle cells appear in blood smear. • Indirect bilirubin level elevated. • Hemoglobin electrophoresis shows majority hemoglobin S (80 to 98 percent). INTERPRETING TEST RESULTS

10. During acute episodes pain control, hydration, and oxygenation are the focus of treatment. The underlying cause that sent the patient into crisis will also need to be treated concurrently. • Administer analgesics to alleviate the pain associated with the vaso-occlusive process: • narcotic pain control necessary when pain is severe • Warm compresses on joint. • Blood transfusion of packed RBC when anemia indicates. • Supplemental oxygen if hypoxic. • Adequate hydration, using IV fluids. • Treat infections. TREATMENT

11. Based on the assessment data, potential complications may include: • Hypoxia, ischemia, infection, and poor wound healing leading to skin breakdown and ulcers • Dehydration • Cerebrovascular accident (CVA, brain attack, stroke) • Anemia • Renal dysfunction • Heart failure, pulmonary hypertension, and acute chest syndrome • Impotence • Poor compliance • Substance abuse related to poorly managed chronic pain COLLABORATIVE PROBLEMS/ POTENTIAL COMPLICATIONS

12. • Increase fluid intake. • Monitor IV fluids. • Monitor pain control. • Record fluid intake and output to monitor renal function. • Administer supplemental O2 to increase available oxygen. • Explain to the patient: • Avoid the cold. • No cold compresses. • Plan for rest periods during the day. NURSING INTERVENTION

13. Based on the assessment data, major nursing diagnoses for the patient with sickle cell crisis may include: • Acute pain related to tissue hypoxia due to agglutination of sickled cells within blood vessels • Risk for infection • Risk for powerlessness related to illness-induced helplessness • Deficient knowledge regarding sickle crisis prevention NURSING DIAGNOSES

14. Hemolytic anemias can result from exposure of the RBC to antibodies. Alloantibodies (i.e, antibodies against the host, or “self”) result from the immunization of an individual with foreign antigens (eg, the immunization of an Rh-negative person with Rh-positive blood). The most common type of alloimmune hemolytic anemia in adults results from a hemolytic transfusion reaction . 2:IMMUNE HEMOLYTIC ANEMIA

15. Autoantibodies are developed by an individual for varying reasons. In many instances, the person’s immune system is dysfunctional, so that it falsely recognizes its own RBCs as foreign and produces antibodies against them. Another mechanism is a deficiency in suppressor lymphocytes, which normally prevent antibody formation against a person’s own antigens.

16. Autoimmune hemolytic anemias can be classified based on the body temperature involved when the antibodies react with the RBC antigen. Warm-body antibodies bind to RBCs most actively in warm conditions (37°C). cold-body antibodies react in cold (0°C). classification

17. The hemolysis may be very mild, so that the patient’s marrow compensates adequately and the patient is asymptomatic. Most patients complain of fatigue and dizziness. Splenomegaly is the most common physical finding, occurring in more than 80% of patients; hepatomegaly, lymphadenopathy , and jaundice are also common. Clinical Manifestations

18. The laboratory tests show a low hemoglobin level and hematocrit. RBCs appear abnormal; spherocytes are common. serum bilirubin level is elevated, and if the hemolysis is severe, the haptoglobin level is low or absent. The Coombs test (also referred to as the direct antiglobulin test [DAT]), which detects antibodies on the surface of RBCs, shows a positive result. Assessment and Diagnostic Findings

19. high doses of corticosteroid s (1 mg/kg per day) until hemolysis decreases. In severe cases, blood transfusions may be required. If neither corticosteroid therapy nor Splenectomy is successful, immunosuppressive agents may be administered. Immunoglobulin administration If corticosteroids or immunosuppressive agents are used, the taper must be very gradual to prevent a rebound “hyperimmune” response and exacerbation of the hemolysis. For patients with cold-antibody hemolytic anemia, treatment may not be required, other than to advise the patient to keep warm; relocation to a warm climate may be necessary. Medical Management

20. It can be difficult to cross-match blood when antibodies are present. If imperfectly cross-matched RBCs must be transfused, the nurse begins the infusion very slowly (10 to 15 mL over 20 to 30 minutes) and monitors the patient very closely for signs and symptoms of a hemolytic transfusion reaction NURSING ALERT

21. Iron deficiency anemia typically results when the intake of dietary iron is inadequate for hemoglobin synthesis. The body can store about one fourth to one third of its iron, and it is not until those stores are depleted that iron deficiency anemia actually begins to develop. Iron deficiency anemia is the most common type of anemia in all age groups, and it is the most common anemia in the world. 3:IRON DEFICIENCY ANEMIA

22. bleeding (from ulcers, gastritis, inflammatory bowel disease, or gastrointestinal tumors). menorrhagia (excessive menstrual bleeding) and pregnancy with inadequate iron supplementation. Patients with chronic alcoholism often have chronic blood loss from the gastrointestinal tract. Other causes include iron malabsorption, as is seen after gastrectomy or with celiac disease. Causes:

23. • Weakness due to anemia and tissue hypoxia • Pallor due to decreased amount of oxygen getting to surface tissues • Fatigue due to anemia and hypoxemia • Koilonychia —thin, concave nails raised at edges, also called spoon nails • Tachycardia and tachypnea on exertion due to increased demand for oxygen Clinical Manifestations

24. • Decrease in serum hemoglobin as fewer RBCs are made. • Serum ferritin is low. • Mean corpuscular volume (MCV) initially normal, then low— microcytic anemia. • Mean corpuscular hemoglobin (MCH) initially normal, then low— hypochromic anemia. • Serum iron level is low. • Serum iron-binding capacity is increased. • Transferrin saturation decreases. • Peripheral blood smear shows poikilocytosis (red blood cells of different shapes). • Platelet count may increase. Assessment and Diagnostic Findings

25. Except in the case of pregnancy, the cause of iron deficiency should be investigated. oral iron preparations—ferrous sulfate, ferrous gluconate, and ferrous fumarate. intravenous or intramuscular administration of iron dextran. Increase dietary intake of iron Medical Management

26. • Monitor intake and output. • Monitor vital signs for tachycardia or tachypnea. • Monitor for reactions to parenteral iron therapy. • Explain to the patient: • Check for bleeding. • Increase iron in diet. • Teach dietary sources of iron. Nursing Management

27. In the anemias caused by deficiencies of vitamin B12 or folic acid, identical bone marrow and peripheral blood changes occur, because both vitamins are essential for normal DNA synthesis. In either anemia, the RBCs that are produced are abnormally large and are called megaloblastic RBCs. 4:MEGALOBLASTIC ANEMIAS (Vitamin B12 & folic acid deficiency anemias)

28. Folic acid, a vitamin that is necessary for normal RBC production, is stored as compounds referred to as folates . The folate stores in the body are much smaller than those of vitamin B12, and they are quickly depleted when the dietary intake of folate is deficient (within 4 months). Pathophysiology FOLIC ACID DEFICIENCY

29. people who rarely eat uncooked vegetables. Alcohol increases folic acid requirements, patients with chronic hemolytic anemias In women who are pregnant Some patients with malabsorptive diseases of the small bowel Folate deficiency occurs

30. intrinsic factor is normally secreted by cells within the gastric mucosa; normally it binds with the dietary vitamin B12 and travels with it to the ileum, where the vitamin is absorbed. Without intrinsic factor, orally consumed vitamin B12 cannot be absorbed, and RBC production is eventually diminished. VITAMIN B12 DEFICIENCY

31. can occur in several ways. Inadequate dietary intake is rare but can develop in strict vegetarians who consume no meat or dairy products. Crohn’s disease ileal resection or gastrectomy. absence of intrinsic factor A deficiency of vitamin B12

32. • Pallor due to anemia • Weakness and fatigue due to anemia • Tingling in hands and feet—“stocking-glove paresthesia”—due to bilateral demyelination of dorsal and lateral columns of spinal cord nerves • Diminished vibratory and position sense • Poor balance due to effect on cerebral function • Dementia appears later in the disease • Atrophic glossitis—beefy red tongue • Nausea may lead to anorexia and weight loss • Premature graying of hair Symptoms of folic acid and vitamin B12 deficiencies are similar, and the two anemias may coexist. However, the neurologic manifestations of vitamin B12 deficiency do not occur with folic acid deficiency, and they persist if B12 is not replaced. HALLMARK SIGNS AND SYMPTOMS

33. • Decreased hemoglobin due to decreased production of RBCs. • Increased MCV— macrocytic anemia. • Positive Schilling test due to decrease in intrinsic factor. • Decreased amount of hydrochloric acid in the stomach (hypochlorhydria) due to changes within the parietal cells of the gastric mucosa. • Positive Romberg test due to ataxia and neurologic changes. • Diminished sensation when testing for vibration, position sense, or propioception of extremities. INTERPRETING TEST RESULTS

34. Lifelong replacement with vitamin B12 will correct the anemia and improve the neurologic changes that have occurred. Initially the patient is given weekly injections of B 12 to combat the deficiency. The injections eventually become monthly for lifelong maintenance. Oral supplementation is not effective in these patients because they cannot adequately absorb vitamin B12 due to insufficient intrinsic factor. • Administer vitamin B 12 by IM injection. • Transfusion of packed RBC if anemia is severe. Medical management

35. Folate deficiency is treated by increasing the amount of folic acid in the diet and administering 1 mg of folic acid daily. Folic acid is administered intramuscularly only for people with malabsorption problems . Cont….

36. Even when the anemia is severe, RBC transfusions should not be used because the patient’s body has compensated over time by expanding the total blood volume. Administration of blood transfusions to such patients, particularly those who are elderly or who have cardiac dysfunction, can precipitate pulmonary edema. If transfusions are required, the RBCs should be transfused slowly, with careful attention to signs and symptoms of fluid overload. NURSING ALERT

37. 5:Aplastic anemia The bone marrow stops producing a sufficient amount of RBC, WBC, and platelets, thereby increasing the risk of infection and hemorrhage. The red cells remaining in circulation are normal in size and color.

38. The cause may also be unknown or idiopathic. This may be due to chemical exposure, high dose radiation exposure, exposure to toxins. Cancer treatments such as radiation therapy and chemotherapeutic agents may suppress bone marrow function, which will result in anemia (low RBC), thrombocytopenia (low platelets), and leukopenia (low WBC). Causes:

39. The bone marrow dysfunction may be slow-onset or sudden. The lifespan of the RBC is longer than the platelets and WBC, so the anemia may show up later than the effects of losing the other cells. Some exposures to toxic agents or medications are severe and potentially fatal in susceptible individuals. PROGNOSIS

40. • Fatigue due to hypoxemia • Weakness due to tissue hypoxia • Pallor due to lack of oxygen reaching superficial tissues due to anemia • Infections due to low white blood cell production, causing decreased ability to fight infection • Bruising ( ecchymosis ), and tiny subcutaneous (SC) hemorrhages ( petechiae ) due to decrease in platelets, altering clotting ability • Bleeding from mucous membranes (GI tract, mouth, nose, vagina) HALLMARK SIGNS AND SYMPTOMS

41. • Low hemoglobin. • Low hematocrit. • Low RBC count. • Thrombocytopenia—low platelet count. • Leukopenia—low WBC. • Reticulocyte count low. • Positive fecal occult blood test. • Decreased cell counts in bone marrow biopsy as body stops producing. INTERPRETING TEST RESULTS

42. • Administer hematopoietic growth factor to correct anemia in patients with low erythropoietin levels: • erythropoietin by SC injection • Administer human granulocyte colony-stimulating factor (G-CSF) to correct low WBC levels: • Packed RBC transfusions when anemia is symptomatic. • Platelet transfusion for severe bleeding. • Bone marrow transplant replaces functioning stem cells. • Administer immunosuppressive drugs,, and corticosteroids. • Splenectomy when spleen is enlarged and destroying RBCs. TREATMENT

43. • Monitor vital signs for changes. • Record intake and output of fluids. • Protect patient from falls. • Avoid IM injections due to altered clotting ability. • Explain to the patient: • No aspirin due to effect on platelet aggregation (clotting ability). • Plan to take rest periods during activities due to fatigue. • Only use an electric razor to decrease risk of bleeding due to decreased platelet count. • Call your physician, nurse practitioner, or physician assistant for signs of bleeding or bruising. NURSING INTERVENTION

44. Polycythemia refers to an increased volume of RBCs. It is a term used when the hematocrit is elevated (to more than 55% in males, more than 50% in females). Dehydration (decreased volume of plasma) can cause an elevated hematocrit, but not typically to the level to be considered polycythemia. 6: Polycythemia

45. Polycythemia is classified as either primary or secondary. 1:POLYCYTHEMIA VERA Polycythemia vera, or primary polycythemia, is a proliferative disorder in which the myeloid stem cells seem to have escaped normal control mechanisms. Classifications:

46. The RBC elevation is predominant; the hematocrit can exceed 60%. This phase can last for an extended period (10 years or longer). The spleen resumes its embryonic function of hematopoiesis and enlarges. the bone marrow may become fibrotic, with a resultant inability to produce as many cells (“burnt out” or spent phase).

47. Secondary polycythemia is caused by excessive production of erythropoietin. This may occur in response to a reduced amount of oxygen, which acts as a hypoxic stimulus, as in cigarette smoking, chronic obstructive pulmonary disease, or cyanotic heart disease, or in non pathologic conditions such as high altitude 2:SECONDARY POLYCYTHEMIA

48. • Facial skin and mucous membranes dark and flushed (plethora) • Hypertension due to increased peripheral vascular resistance and thickening of the blood • Itching worse after warm shower due to histamine • Headache and difficulty concentrating • Vision blurred, tinnitus (ringing in ears), and hearing changes • Thrombosis due to vascular stasis • Spleen enlargement (splenomegaly) • Tissue hypoxia and possible infarction of heart, spleen, kidneys, and brain due to thrombosis Clinical manifestations:

49. • Increased RBC count. • Increased hemoglobin. • Increased hematocrit level. • Increased WBC count. • Increased platelet count. • Increased uric acid level. • Increased potassium. • Increased vitamin B 12 level. • Bone marrow panhyperplasia ; iron stores absent. INTERPRETING TEST RESULTS

50. Treatment is aimed at maintaining bloodflow to the smaller vessels and diminishing the amount of excess blood cells being made by the bone marrow. • Periodic scheduled phlebotomy—the removal of 500 ml of blood—to reduce the hematocrit level to below 45; may be done weekly. TREATMENT

51. • Adequate hydration. • Anticoagulants such as aspirin. • Administer myelosuppressive medication: • Administer medication to lower uric acid • Radiation therapy. • Antihistamine for pruritis. Management of secondary polycythemia may not be necessary; when it is, it involves treating the primary problem. Cont….