Management of Clients with Hematologic Disorders

1. Management of Clients with Hematologic Disorders NRS 108 Majuvy L. Sulse RN, MSN, CCRN

2. HEMATOLOGIC DISORDERS • Disorders associated with Erythrocytes • Disorders of bleeding • Disorders associated with white blood cells • Lymphomas

3. ANEMIA (decreased in number of RBCs) Low Hgb H/H, Bone marrow aspiration, Peripheral smear ( Diagnosis) Abnormal & deficient production, blood loss, destruction of RBC ( Causes) Less 02 carrying capacity Hypoxia Pallor, Fatigue, Palpitation, Low BP, SOB, DOE, MI, Renal failure

4. Management of Anemia • Medical: • Identify cause • Treat cause • Relieve symptoms • Prevent complications • Nursing: • Assess • Educate

5. Classification of Anemia • Based on the size of the RBC • Normocytic • Microcytic • Macrocytic

6. Iron Deficiency Anemia • Causes: • Inadequate iron supply • Chronic blood loss without iron replacement • Decreased iron absorption in the intestines • Signs/ Symptoms: • Hypochromic (low MCH), microcytic ( low MCV) • Elevated serum binding capacity • Brittle, spoon-shaped nails with longitudinal ridges • Cheilosis (painful mouth cracks/ sores) • Red shiny tongue • Insidious development of fatigue

7. Diagnosis/Treatment • Treatment: • Identify cause • Diagnosis: • Laboratory values • Gastroscopy • Sigmoidoscopy • Occult blood in stools • Radiographic studies of GI • Iron supplement • Nutritional/dietary

8. Megaloblastic Anemia • Predominance of megaloblasts & lack of normoblasts • Includes pernicious anemia, Vit. B12 & Folic acid deficiencies • Related to surgery particularly of terminal ileum where B12 is absorbed; vegetarian diet; prolonged exposure to nitrous oxide • Related to aging & long term gastritis • Related to alcohol malnutrition & malabsorption (Folic acid deficiency) • Macrocytic, normochromic RBC • Lack of Intrinsic factor • Autoimmune response

9. Diagnosis & Treatment: (Megaloblastic Anemia) • Schilling test- definitive dx of pernicious anemia • Gastric secretion analysis- pH, free HCL, low gastric secretion

10. Diagnosis & Treatment :(Megaloblastic Anemia) • Management- • Lifelong tx with Vit B12 injection • Iron & Folic acid supplement • Nutritional/ dietary changes • Foods that are rich in folic acid and vitamin B12 include the following: • eggs • meat • poultry • milk • shellfish • fortified cereals

11. Aplastic Anemia • Low Hb & pancytopenia • Unknown etiology / autoimmune disturbance • Direct injury by Myelotoxins- agents causing bone marrow damage when received in large doses • Medication induced: • Chemo, chloramphenicol, sulfonamides, mephenytoin, quinine • Exposure to environmental hazards: • Benzene, insecticides, radiation & radioactive materials • Infections • Hepatitis, miliary TB, EPBV • Congenital/hereditary causes

12. Aplastic Anemia-Manifestations & Dx • Manifestations- • Exertional dyspnea, fatigue, pallor, infections • Bleeding (nasal,oral, rectal,vaginal) ecchymosis, petechiae, pupura • Low platelets (less 30, 000), RBCs, WBCs • Dry bone marrow on aspiration • Diagnosis- • Differential count • Manifestations • History of exposure to myelotoxins

13. Aplastic Anemia • Medical • Remove causative • Idiopathic cause- treat with steroids, hormone therapy • Autoimmune cause- bone marrow transplants (younger than 30 or those who have not received transfusions yet • Antithymocyte & cyclosporine therapy- skin testing & watch for allergic reactions • Blood transfusions • Frequent CBCs esp for those on radiation therapy • Nursing management • Infection control • Client education

14. Anemia from Blood Loss • Types • Acute-trauma, complications from surgery • Hypovolemia, hypotension, hypoxemia, weakness, tachycardia, stupor • Chronic- bleeding ulcer, hemorrhoids • Gradual and vague symptoms as fatigue, pallor, dyspnea • Medical/ Nursing Interventions • Identify source of bleeding • Control through medical or surgical interventions • Blood transfusions

15. Hemolytic Anemia • Abnormal destruction of RBCs by • Intrinsic-defective RBCs, enzyme deficit(G6PD) • extrinsic factors- toxins, injury as in prosthetic heart valves • Failure of bone marrow to replace destroyed RBCs • Sites of hemolysis: • Intravascular-circulation • Extravascular- macrophages of spleen, liver & bone marrow

16. Findings Normocytic anemia Reticulocytosis as compensatory mechanism Increased RBC fragility Short lifespan Hyperbilirubinemia- blood, urine, stools S/S as in Anemia Treatment Identify/Treat cause IV fluids to flush kidneys NAHCO3 or Na Lactate to alter urine pH (decrease) precipitation in renal tubules Splenectomy Findings/ Treatment

17. Sickle Cell Anemia and Sickle Cell Trait • Inherited, autosomal, recessive disorders of HB synthesis resulting in decrease O2 to the tissues (hypoxia) and obstruction of blood vessels • Substitution of valine for glutamic acid in B-globin gene • Primarily seen in the black population • Sickle cell trait is a mild form of the disease and it is the commonest • Sickle cell trait is prevalent in Africa • Resistant to the parasite that causes malaria • Genetically inherited from each parent

18. Inheritance

19. Pathophysiology • Sickling of RBC-triggered by • Hypoxia- low O2 tension in blood • High altitude • Emotional/ physical stress • Surgery • Blood loss • Infection-bacterial & viral • Dehydration • Acidosis • Decreased plasma volume- Increased blood viscosity • Hypothermia • Stress

20. Pathophysiology • Sickled cell-rigid & elongated causes tissue injury (as cells can’t pass through small vessels) results in local hypoxia anemia as more cells are hemolyzed by spleen • Initially reversible with re-oxygenation then becomes irreversible due to cell membrane damage from recurrent sickling

21. Sickle Cell crisis • Exacerbation of RBC sickling • Vaso- occlusion (Vaso-occlusive crises) - vasospasm • Changes in membrane permeability plasma loss & hemoconcentration development of thrombi, tissue ischemia, infarction, necrosis • Shock is a life threatening consequence • Frequency, extent, & severity of episode is dependent on percentage of HbS present

22. Causes of Sickle Cell Crisis • Vaso - Occlusion • Aplastic crisis • Hemolytic crisis • Sequestration crisis • Mixed crisis

23. Types of Sickle Cell Disease • Sickle cell anemia • Sickle cell Thalassemia • Sickle cell HbC-

24. Clinical Manifestations • Cardiovascular changes • Skin changes • Abdominal changes • Musculoskeletal changes • Central nervous system changes

25. Clinical Manifestation • Noticed after 6 months when fetal Hb is no longer present • Improper growth related to anemia • Retarded growth and delay in sexual maturity • Hand- foot syndrome- edema of hands and feet • Pain from tissue ischemia-hands, feet , joints

26. Clinical Manifestation • Weakness and fatigue-exercise intolerance • Jaundice- development of gallstones • Pallor-of mucous membrane-grayish cast on skin • Priapism-occlusion of penile veins • Infarct on the spleen - small and scarred • Leg ulcers in about 75% of cases

27. CHF from ischemia & heart enlargement Acute Chest syndrome-fever, chest pains, cough, dyspnea Pulmonary infarct- pulmonary HTN, MI, Cor Pulmonale Blindness from retinal obstruction-hemmorhage-detachment Renal failure- hemoconcentration Autosplenectomy-shrinking of spleen from repetitious scarring CVA- thrombus formation Osteoporosis/ Osteosclerosis Leg ulcers esp. at ankles-tissue hypoxia Infection- absence of phagocytic activity by spleen Complications

28. Diagnostic findings • Peripheral Smear • Sickling Test • DNA • Elevated serum bilirubin levels • Skeletal xrays-reveal bone/joint deformities & flattening • MRI-check for clots (CVA)

29. Medical/ Nursing Management • No cure • Supportive interventions: pain relief, • Hydration, O2, rest, blood transfusions • Patient Teaching to avoid factors that cause crisis • Assessment of family understanding of the disease & coping mechanism • Links for support • Genetic counseling

31. Get Involved Class! • What nursing diagnosis should receive the highest priority in a client with sickle cell crisis? • A nurse is preparing a teaching plan for a sickle cell client, what should the nurse emphasize on to prevent sickle cell crisis?

32. Polycythemia Vera • Excessive production of erythrocytes, leukocytes, platelets due to excessive activation of pluripotent stem cells • Manifestations • HTN- headache, vertigo, tinnitus, dizziness, visual disturbances • CHF- angina- hypervolemia & viscosity • Intermittent claudication- thrombophlebitis

33. Polycythemia Vera • Stroke- thrombi formation • Pruritus- histamine release from basophils • Hemmorrhage- vessel rupture from tissue distention • Hepatomegaly & Slenomegaly from organ engorgement • Plethora- Ruddy complexion • Gout—hyper uric acid production from RBC destruction

34. Diagnosis • RBC=8-12 million • Hgb=18-25 gm • Increased platelet count • HCT= >54% in men; 49% in women • Elevated WBCs with basophilia • Increased serum uric acid, B12 • Splenomegaly • Hyperplastic bone marrow

35. Medical/Nursing Management • Goal of treatment is • Reduce blood volume & viscosity • Phlebotomy to Hct levels of 45-48% (about 300-500ml/day) • Reduce bone marrow activity- myelosuppressive agents & radioactive phosphorous • Hydration therapy with I & O

36. DISORDERS OF BLEEDING- THROMBOCYTOPENIA • Decreased production of platelets below 150,000/uL • Manifests as bleeding- skin bruises easily • Maybe • acquired –food, drugs, infections, aplastic anemia • inherited- pancytopenia, hereditary thrombocytopenia

37. Diagnosis/ Management • CBC shows low platelet & Hb count • Assess for hx of NSAIDS • Avoid injury • Good oral hygiene & skin care • Rectal enemas & suppository-avoid constipation • Avoid IM, SC injections, rectal temperatures • Apply pressure on any bleeding source • Monitor signs of bleeding

38. Immune Thrombocytopenia Purpura- ( ITP) • Autoimmune bleeding disorder • Platelets coated with antibodies • Destroyed by macrophages in liver & spleen • Survival is 1-3 days instead of 8-10 • Gradual onset & transient remissions occur

39. Clinical Manifestations • Petecchiae- small, flat, pinpoint red • Purpura-numerous petecchiae • Ecchymosis-large purplish lesions • Epistaxis- • Bleeding gums • Heavy menses • Complication=hemorrhage

40. Diagnosis • Platelet Ct<100, 000 • Prolonged bleeding time with normal coagulation time • Increased capillary fragility • Positive platelet antibody test • Bone marrow aspirate contains normal or increased megakaryocytes

41. Management • Cortecosteroids (prednisone)-suppreses the phagocytic response of Spleenic macrophages, depress antibody formation, reduce capillary permeability & bleeding time • IV immunoglobulin (IVIG) • Immunosuppressive therapy • Splenectomy- • Platelet transfusions

42. Hemophilia • Characterized by prolonged bleeding after surgical/ dental or small trauma or cuts • Types • Hemophilia A- factor Vlll-most common-80% cases • Hemophilia B- factor lX deficiency-inherited gene • Von Willebrand’s disease- deficient Vlll & platelet dysfunction

43. Etiology • Sex linked genetic disorder • Transmitted by females but males express the disorder • Carriers transmit the gene to half their daughters and the disorder to half their sons • Males transmit the gene to all their daughters but none to their sons

44. Clinical Manifestations • Slow persistent bleeding from cuts/scratches • Delayed hemorrhage-hours/days after injury • Severe bleeding after dental surgery • GI bleed • Nosebleed • Hematoma • Prolonged APTT

45. Treatment • Goal is to stop bleeding • Increase anti-hemophilic factor-give factor Vlll, lX • Prevent complications • Support therapy

46. LEUKEMIA • DESCRIPTION • Malignant exacerbation in the number of leukocytes, usually at an immature stage, in the bone marrow • May be acute, with a sudden onset and short duration, or chronic, with a slow onset and persistent symptoms over a period of years • Affects the bone marrow, causing anemia, leukopenia, the production of immature cells, thrombocytopenia, and a decline in immunity

47. LEUKEMIA • DESCRIPTION • The cause is unknown and appears to involve gene damage of cells leading to the transformation of cells from a normal state to a malignant state • Risk factors include genetic, viral, immunological, and environmental factors and exposure to radiation, chemicals, and medications

48. CLASSIFICATION OF LEUKEMIA • ACUTE LYMPHOCYTIC LEUKEMIA (ALL) • Mostly lymphoblasts present in bone marrow • Age of onset is less than 15 years • CNS manifestation common-leukemic meningitis • Normally, the lymphocytes fight infection by making antibodies that attack harmful elements. But, in ALL, the cells are immature and overabundant. They crowd out other blood cells, and may collect in the blood, bone marrow, and lymph tissue.

49. CLASSIFICATION OF LEUKEMIA • ACUTE MYELOGENOUS LEUKEMIA (AML) • affects the young blood cells (called blasts) that develop into a type of white blood cell (called granulocytes). The main function of granulocytes is to destroy bacteria. The blasts, which do not mature and become too numerous, remain in the bone marrow and blood. • Age of onset is between 15 and 39 years

50. Classification • CHRONIC MYELOGENOUS LEUKEMIA (CML) • Mostly granulocytes present in bone marrow • Age of onset is after 50 years of age • CML occurs mainly in adults and is rare in children • CHRONIC LYMPHOCYTIC LEUKEMIA (CLL) • Mostly lymphocytes (B cells) present in bone marrow • Age of onset is after 50 years of age,