UNIT-5 AHN-B.pptx, Nursing lecture semester 4

Lecture

1. Hematology Disorders MEHMOOD AHMED NURSING INSTRUCTOR BSN YEAR II SEMESTER III ADULT HEALTH NURSING UNIT V (B) HEMATOLOGY DISORDERS KGH SCHOOL OF NURSING

2. POLYCYTHEMIA Polycythemia is an increased volume of red blood cells. The hematocrit is elevated by more than 55% in men or more than 50% in women. Polycythemia is classified as either primary or secondary .

3. Primary Polycythemia Primary polycythemia, is a proliferative disorder of the myeloid stem cells. The bone marrow is hyper-cellular , and the erythrocyte, leukocyte, and platelet counts in the peripheral blood are elevated. Diagnosis is based on an elevated erythrocyte mass, a normal oxygen saturation level, and often an enlarged spleen. The erythropoietin level may not be as low as would be expected with an elevated hematocrit .

4. Secondary Polycythemia Secondary polycythemia is caused by excessive production of erythropoietin. This may occur in response to a hypoxic stimulus , as in COPD or cyanotic heart disease, or in certain hemoglobinopathies in which the hemoglobin has an abnormally high affinity for oxygen, or it can occur from a neoplasm, such as renal cell carcinoma. Management of secondary polycythemia involves treatment of the primary problem. If the cause cannot be corrected, phlebotomy may be necessary to reduce hypervolemia and hyperviscosity.

5. Clinical Manifestations Patients typically have a ruddy complexion and splenomegaly. The symptoms are due to the increased blood volume headache , dizziness, tinnitus, fatigue, paresthesias ( Paresthesia  is an abnormal sensation of the skin tingling , pricking, chilling, burning, numbness with no apparent physical cause ) and blurred vision or to increased blood viscosity (angina, claudication ( Claudication  is pain and/or cramping in the lower leg due to inadequate blood flow to the muscles), dyspnea, and thrombophlebitis ( thrombophlebitis is an inflammatory process that causes a blood clot to form and block one or more veins, usually in your legs ). Blood pressure and uric acid are often elevated.

6. Medical Management The objective of management is to reduce the high red blood cell mass. Phlebotomy is performed repeatedly to keep the hemoglobin within normal range; iron supplements are avoided. Chemotherapeutic agents are used to suppress marrow function (may increase risk for leukemia). Medicine may be used to inhibit platelet aggregation and control the thrombocytosis related to polycythemia. Interferon alpha-2b (Intron-A) is the most effective treatment for managing the pruritus ( Pruritus  or itch is defined as an unpleasant sensation of the skin that provokes the urge to scratch) associated with polycythemia. Antihistamines may be administered to control pruritus (not very effective). Allopurinol is used to prevent gouty attacks when the uric acid level is elevated.

7. Nursing Management Assess risk factors for thrombotic complications and teach patient to recognize signs and symptoms of thrombosis. Discourage sedentary behavior, crossing the legs, and wearing tight or restrictive clothing (particularly stockings) to reduce the likelihood of DVT. Advise patient to avoid aspirin and medications containing aspirin (if patient has a history of bleeding). Advise patient to minimize alcohol intake and avoid iron and vitamins containing iron. Suggest a cool or tepid bath for pruritus, along with cocoa butter–based lotions and bath products to relieve itching.

8. LEUKEMIA Greek word leukos means white, and haima means blood. It is a type of cancer of blood or bone marrow characterized by an abnormal increase of immature WBCs. Leukemia is a broad term covering a spectrum of diseases.

9. Classification of leukemia Acute Leukemia Rapid production of blood cells Malignancy of cells of bone marrow. Common in children. Chronic Leukemia Excessive production of relatively mature, but still abnormal white blood cells. It occurs in old age people. it is mylogenous leukemia (granulocytes) or lymphatic leukemia (increase lymphocytes).

10. Sign & symptoms Petechia (red or purple spot on the body). Bleeding (increase WBCs production decrease platelets). Frequent infections (Diarrhea, pneumonia, tonsillitis). Anemia leads to pallor. Fatigue. Enlarge spleen. Headache

11. Causes Mutation in DNA. Ionizing radiation (X-rays). The Human T- lymphotropic virus Chemicals (benzene and chemotherapy).

12. Labs Viral culture Antibody tests for screening and diagnosis. CBC Biopsy

13. Treatment Antiviral, such as acyclovir Oral therapy may be episodic, whenever the first signs of a recurrence are recognized, or continuous to suppress recurrent infection. I.V. administration (acyclovir) may be necessary for severe infections. Prednisone (corticosteroid drug effective immunosuppressant). Vincristine (used in cancer chemotherapy). Encourage fluid intake. Restoring Skin Integrity Administer antiviral agent and teach patient its proper use and adverse effects.

14. HODGKIN LYMPHOMA It is a cancer of the lymphatic system, which is part of your immune system . (HD) is a malignant disorder of lymphoreticular system; a lymphoreticular neoplasm primarily of B cell lineage involving lymph nodes and the lymphatic system has unique molecular, histologic, immune phenotypic and clinical features.

15. Causes Variation in the incidence of HD in different ethnic groups and association with human leukocyte antigen suggests that inherited susceptibility plays an important role in the pathogenesis. Environmental factors such as Epstein-Barr virus infection, Familial clustering of cases and Higher incidence in twins may be some of the other contributing factors.

16. Sign and Symptoms Lymphadenopathy , usually in the cervical, supraclavicular, and mediastinal areas; mediastinal presentation common in adolescents and young adults; (significant mediastinal adenopathy may cause cough, dyspnea, or superior vena cava syndrome) Painless , movable lymph nodes in tissues surrounding involved area Unexplained fever Weight loss Drenching night sweats Malaise Painless cervical or supraclavicular lymphadenopathy

17. Staging in Lymphoma

18. Diagnosis Complete blood count—diagnostic (anemia may indicate advanced disease) Erythrocyte sedimentation rate (ESR)—may be elevated at diagnosis Serum copper, iron, calcium, and alkaline phosphatase levels—also may be elevated at diagnosis Liver and renal function tests—to assess organ involvement Urinalysis—to determine renal involvement Chest radiographic study—to determine mediastinal or hilar node involvement Computed tomography—to evaluate mediastinal, pulmonary, and abdominal disease Gallium and/or positron emission tomography (PET) scan to determine the extent of involvement Excisional lymph node biopsy—essential to diagnosis and staging Bone marrow biopsy if patient has stage 3 or 4 disease according to imaging studies

19. Treatment Treatment modalities have varied from total nodal radiation therapy to chemotherapy to combination of chemotherapy and radiotherapy with significant improvement in survival rate throughout the last three decades. All children generally receive combination chemotherapy as initial treatment .

20. NON-HODGKIN LYMPHOMA Non-Hodgkin’s Lymphoma (NHL) is neoplasm of a wide range of cell types that comprise the immune system. NHL most commonly occurs during the second decade of life and occurs less frequently in children less than three years of age. Pediatric NHL are high grade, diffuse and aggressive with propensity for dissemination Reed-Sternberg cells are large, abnormal lymphocytes (a type of white blood cell) that may contain more than one nucleus. These cells are found in people with Hodgkin lymphoma .

21. B cells B cells fight infection by producing antibodies that neutralize foreign invaders. Most non-Hodgkin's lymphoma arises from B cells. T cells T cells are involved in killing foreign invaders directly. Non-Hodgkin's lymphoma occurs less often in T cells

22. Sign and Symptoms Intra-abdominal Involvement Possible symptoms mimicking appendicitis (pain, right lower quadrant tenderness) Ovarian , pelvic, retroperitoneal masses Ascites Vomiting Diarrhea Weight loss

23. Sign and Symptoms Mediastinal Involvement Pleural effusion Tracheal compression Superior vena cava syndrome Coughing , wheezing, dyspnea, respiratory distress Edema of upper extremities Mental status changes Primary Nasal, Paranasal, Oral, and Pharyngeal Involvement Nasal congestion Epistaxis Headache Irritability

24. Staging in NHL

25. Diagnosis Bone marrow biopsy—to identify malignant cells with bone marrow involvement Lumbar puncture—to determine presence of malignant cells in CNS Complete blood count—diagnostic for bone marrow dysfunction; may show elevated white blood cell count, decreased hemoglobin level, hematocrit, and platelet count Liver and kidney function tests—liver function test values may be elevated with liver involvement; kidney function test values may be elevated with kidney involvement Lactate dehydrogenase level—elevated owing to tumor lysis Serum uric acid level—elevated owing to cellular tumor load Epstein-Barr virus test—positive result has been associated with NHL Bone scan—to determine the presence of metastases in the bone Chest radiograph—to determine the presence of metastases in the lung Computed tomography and magnetic resonance imaging— to determine the presence of metastases in other areas of the body

26. Treatment Childhood NHL is an extremely chemosensitive disease. Surgery plays a very limited role, mainly for arriving at a diagnosis. Radiation of primary sites is used very rarely in emergency situations.

27. AUTOIMMUNE AND THROMBOTIC THROMBOCYTOPENIC PURPURA Autoimmune Thrombocytopenic Purpura ( ITP) is a bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting. People with the disease have too few platelets in the blood. The peak age is 1-4 yr. ITP seems to occur more often in late winter and spring after the peak season of viral respiratory illness.

28. Pathophysiology An autoantibody directed against the platelet surface develops with resultant sudden onset of thrombocytopenia. In some patients ITP appears to arise in children infected with Helicobacter pylori or rarely following the measles, mumps, rubella vaccine.

29. Clinical Manifestations The classic presentation of ITP is a previously healthy 1-4 yr old child who has sudden onset of generalized petechiae and purpura. Often there is bleeding from the gums and mucous membranes, particularly with profound thrombocytopenia (platelet count <10 × 109/L). 

30. Classification system Depending on the basis of symptoms and signs, but not platelet count; ITP is classified as: Bruising and petechiae –Occasional minor epistaxis Very little interference with daily living Class More severe skin and mucosal lesions – More troublesome epistaxis and menorrhagia  Bleeding episodes—menorrhagia, epistaxis, melena—requiring transfusion or hospitalization - Symptoms interfering seriously with the quality of life 

31. Prognosis Severe bleeding is rare (<3% of cases) In 70-80% of children who present with acute ITP, spontaneous resolution occurs within 6 months Fewer than 1% of patients develop an intracranial hemorrhage. Approximately 20% of children who present with acute ITP go on to have chronic ITP ITP in younger children is more likely to resolve The development of chronic ITP in adolescents approaches 50%.

32. Laboratory Findings Severe thrombocytopenia (platelet count <20 × 109/L) is common, and platelet size is normal or increased, reflective of increased platelet turnover Hemoglobin value, white blood cell (WBC) count, and differential count should be normal. Indications for bone marrow aspiration/biopsy include: An abnormal WBC count or differential Unexplained anemia Findings on history and physical examination suggestive of a bone marrow failure syndrome or malignancy. Other laboratory tests should be performed as indicated by the history and physical examination

33. Diagnosis Autoimmune thrombocytopenia may be an initial manifestation of : Systemic lupus erythematosus  ( SLE ) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs . HIV infection Common variable immunodeficiency Lymphoma(rarely )

34. Treatment Platelet transfusion in ITP is usually contraindicated unless life-threatening bleeding is present (Antiplatelet antibodies bind to transfused platelets as well as they do to autologous platelets) Initial approaches to the management of ITP include the following: No therapy other than education and counseling of the family and patient for patients with minimal, mild, and moderate symptoms, as defined earlier.

35. Treatment Intravenous immunoglobulin (IVIG). IVIG at a dose of 0.8- 1.0 g/kg/day for 1-2 days induces a rapid rise in platelet count (usually >20 × 109/L) in 95% of patients within 48 hr. IVIG appears to induce a response by downregulating Fc-mediated phagocytosis of antibody-coated platelets. IVIG therapy is : Time-consuming to administer After infusion, there is a high frequency of headaches and vomiting, suggestive of IVIG-induced aseptic meningitis. 

36. DISSEMINATED INTRAVASCULAR COAGULATION (DIC) Disseminated intravascular coagulation (DIC) is a potentially life-threatening sign (not a disease itself) of a serious under- lying disease mechanism. DIC may be triggered by sepsis, trauma, cancer, shock, abruption placentae, toxins, or allergic reactions. The severity of DIC is variable, but it is potentially life threatening.

37. Pathophysiology N ormal hemostatic mechanisms are altered so that tiny clots form within the microcirculation of the body. These clots consume platelets and clotting factors, eventually causing coagulation to fail and bleeding to result. This bleeding disorder is characterized by low platelet and fibrinogen levels; pro- longed prothrombin time (PT), partial thromboplastin time (PTT), and thrombin time; and elevated fibrin degradation products (D-dimers). The primary prognostic factor is the ability to treat the underlying condition that precipitated DIC.

38. Clinical Manifestations Clinical manifestations of DIC are primarily reflected in compromised organ function or failure, usually a result of excessive clot formation (with resultant ischemia to all or part of the organ) or, less often, bleeding. Patient may bleed from mucous membranes, venipuncture sites, and gastrointestinal and urinary tracts. Bleeding can range from minimal occult internal bleeding to profuse hemorrhage from all orifices. Patients typically develop multiple organ dysfunction syndrome (MODS), and they may exhibit renal failure as well as pulmonary and multifocal central nervous system infarctions as a result of micro-thromboses, macro-thromboses, or hemorrhages .

39. Assessment and Diagnostic Findings Clinically , the diagnosis of DIC is often established by a drop in platelet count, an increase in PT and activated partial thromboplastin time ( A PTT ), an elevation in fibrin degradation products, and measurement of one or more clotting factors and inhibitors (eg, antit-hrombin [AT]). The International Society on Thrombosis and Hemostasis has developed a highly sensitive and specific scoring system using the platelet count, fibrin degradation products, PT, and fibrinogen level to diagnose DIC. This system is also useful in predicting the severity of the disease and subsequent mortality.

40. Medical Management The most important management issue is treating the underlying cause of DIC. A second goal is to correct the secondary effects of tissue ischemia by improving oxygenation, replacing fluids, correcting electrolyte imbalances, and administering vasopressor medications. If serious hemorrhage occurs, the depleted coagulation factors and platelets may be replaced (cryoprecipitate to replace fibrinogen and factors V and VII; fresh-frozen plasma to replace other coagulation factors ).

41. Nursing Management Maintaining Hemodynamic Status Avoid procedures and activities that can increase intracranial pressure, such as coughing and straining. Closely monitor vital signs, including neurologic checks, and assess for the amount of external bleeding. Avoid medications that interfere with platelet function, if possible (eg, beta-lactam antibiotics, acetylsalicylic acid, nonsteroidal anti-inflammatory drugs). Avoid rectal probes and rectal or intramuscular injection medications. Use low pressure with any suctioning. Administer oral hygiene carefully: use sponge-tipped swabs, salt or soda mouth rinses; avoid lemon- glycerine swabs, hydrogen peroxide, commercial mouthwashes. Avoid dislodging any clots, including those around IV sites, injection sites, and so forth.

42. Monitoring for Imbalanced Fluid Volume Auscultate breath sounds every 2 to 4 hours. Monitor extent of edema. Monitor volume of IV medications and blood products; decrease volume of IV medications if possible. Administer diuretics as prescribed.

43. References Brunner and Suddarth’s textbook of medical-surgical nursing (12th ed .) Philadelphia: Lippincott Williams & Wilkins.