hematology dhe he je join je disorders-2.pptx

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1. Hematology Disorders-2 BY ISHAQ ARSHAD

2. Hematology Disorders-2 7:Leukemia 8:Hodgkin disease & Non Hodgkin lymphoma 9:Autoimmune and thrombotic Thrombocytopenic purpura, 10:DIC

3. 7:LEUKEMIAS Cancers of hematopoietic system are disorders that result from proliferation of malignant cells. Malignant cells are originated in bone marrow, Thymus, and lymphatic tissue. Blood cells that originate in bone marrow are called hematopoietic cells. Blood cells that originate in lymph are called lymphoid cells. Leukemia (Cancer of Bone marrow) Lymphoma (Cancer of lymphoid tissue)

4. DEFINITION Leukemia is a malignant disease of the blood- forming organs. Leukemia is a malignant progressive disease in which the bone marrow & other blood forming organs produce increased no. of immature / abnormal leucocytes, these suppresses the production of normal blood cells, leading to anemia & other symptoms.

5. ETIOLOGY AND RISK FACTORS The exact cause is unknown. Several factors are associated with leukemia include: 1. Genetic factors: – A high incidence of acute leukemias & chronic lymphocytic leukemias is reported in certain families.

6. – Hereditary abnormalities associated with an increased incidence of leukemia are Down’s syndrome, Fanconi’s aplastic anemia etc. – Identical twins, fraternal twins, and siblings of children with leukemia are also at increased risk.

7. 2. Over-exposure to ionizing radiations and chemicals : – Can become a major risk factor for development of leukemia, with disease developing years after initial exposure. – Alkylating agents used to treat other cancers, especially in combination with radiation therapy, increase a person’s risk of leukemia. – Workers exposed to chemical agents, such as benzene (an aromatic hydrocarbon), are at a much higher risk.

8. 3. Congenital abnormalities: – Down’s syndrome. 4. The presence of : – Primary immunodeficiency ,& – Infection with the Human T-cell Leukemia Virus Type-1 (HTLV-1).

9. TYPES There are 4 major types on the basis of acute versus chronic, the term acute and chronic refers to cell maturity and nature of disease onset: 1. Acute lymphocytic leukemia. 2. Acute myelogenous leukemia. 3. Chronic lymphocytic leukemia. 4. Chronic myelogenous leukemia.

10. Primary diff. b/w the four types is the rate of progression and where the cancer develops. Chronic leukemia cells do not mature all the way, so they are not as capable of defending against infections as normal lymphocytes. Acute leukemia cells begin to replicate before any immune functions have developed

11. 1. Acute lymphocytic leukemia: Most common type of leukemia in children, and accounts for 15% in adults. In this type , immature lymphocytes proliferate in bone marrow : most are of B- cell origin. • Age of onset : – Before 14 year of age , – peak incidence in b/w 2-9 years of age, – and in older adults

12. • Clinical manifestations : – Fever, pale skin, bleeding, anorexia, fatigue and weakness. – Bone , joint and abdominal pain. – Generalized lymph- adenopathy , infection, weight loss. – Hepatomegaly , splenomegaly, headache, mouth sores. – Increased ICP (nausea, vomiting, lethargy, cranial nerve dysfunction).

13. • Diagnostic evaluation: – Low RBC count, Hb, Hct, low platelet count. – Low, or high WBC count. – Transverse lines of rarefaction at ends of long bones on X-rays. – Hyper cellular bone- marrow with lymphoblast. – Lymphoblast also possible in CSF. – Presence of Philadelphia chromosomes

14. 2. ACUTE MYELOGENOUS LEUKEMIA: This type represents only 1/4th of all leukemias, in which 85% of this type in adults. It is characterized by uncontrolled proliferation of myeloblasts , precursors of granulocytes. There is hyperplasia of bone marrow.

15. • Age of onset: – Its onset is often abrupt & dramatic, a patient may have serious infections and abnormal bleeding from the onset of disease. – Increase in incidence with advancing age, peak incidence b/w 60 and 70 year of age.

16. • Clinical manifestations: – Fatigue, weakness, headache, mouth sores, anemia, bleeding, fever, infection, sternal tenderness, gingival hyperplasia, minimal hepatospleenomegaly & lymphadenopathy.

17. • Diagnostic evaluation: – low RBC count, Hb, Hct, Low platlet count, low to high WBC count with myeloblasts . – high LDH, greatly hypercellular bone marrow with myeloblasts .

18. 3. CHRONIC LYMPHOID LEUKEMIA: It is most common type in adults. It is characterized by production and accumulation of functionally inactive but long lived, small, mature- appearing lymphocytes. The type of lymphocytes involved is usually Bcell . The lymphocytes infiltrate the organ.

19. Lymphadenopathy is present throughout the body , and there is an increased incidence of infection because of T-cell deficiencies . Pressure on nerves from enlarged lymph nodes cause pain and even paralysis. • Onset: – 50- 70 years of age, rare below 30 year of age.

20. • Clinical mafestation : – No symptoms frequently. – Disease is often detected during examination for unrelated conditions, chronic fatigue, anorexia. – Splenomegaly & lymphadenopathy. – Hepatomegaly, may progress to fever, night sweats, weight loss.

21. Diagnostic evaluation: – Mild anemia and thrombocytopenia with disease progression; total WBC count >100,000 /micro L. – Hemolytic anemia (4-11%). – Thrombocytopenia purpura (2-4%). – Hypo- gammaglobulinemia

22. 4.CHRONIC MYELOGENOUS LEUKEMIA: It is caused by excessive development of mature neoplastic granulocytes in the bone marrow. The excess neoplastic granulocytes move into peripheral blood in massive numbers and ultimately infiltrate the liver & spleen. These cells contain a distinctive cytogenetic abnormality, the Philadelphia chromosomes, which serves as a disease marker and results from translocation of genetic material between chromosomes 9 and 22.

23. • Age of onset: – 25 to 60 year of age, peak incidence around 45 year of age. • Clinical manifestations: – No symptoms are seen earlier. – Fatigue and weakness, fever, sternal tenderness, weight loss, joint pain , pain in bone, massive splenomegaly, increase in sweating.

24. • Diagnostic findings: – Low RBC’s count, Hb, Hct. – High platlet count early, lower count later. – Presence of Philadelphia chromosomes in 90% 0f patients

25. CLINICAL MANIFESTATION • Clinical features of leukemia relate to the problems caused by bone marrow failure and formation of leukemic infiltrates. • As leukemia progresses : – Fewer normal blood cells are produced. – Abnormal WBC’s continue to accumulate ( don’t go through apoptosis).

26. – The leukemic cells infiltrate the patient’s organs, leading to problems such as splenomegaly, hepatomegaly, lymphadenopathy, bone pain, meningeal irritation and oral lesions. – Solid masses resulting from collection of leukemic cells called chlormas can also occur.

27. DIAGNOSTIC EVALUATION • A history collection. • Physical examination. • Peripheral blood evaluation ( WBC & platelet count). • Bone marrow examination (sampling from hip bone). • Lumbar puncture and CT scan are done to determine the presence of leukemic cells outside of blood & bone marrow.

28. MANAGEMENT PHARMACOLOGICAL MANAGEMENT : It includes the chemotherapy , which is a major form of treatment for leukemia. This drug treatment uses chemicals to kill leukemia cells. This can be a drug or a combination of drugs. these can be in form of pill or IV injection.

29. • Administer an anthracycline • Administer platelet transfusions. • Administer Filgrastim for neutropenia. • Administer antibiotics for infections. • Administer immunosuppressive to avoid transplant rejection

30. • Administer Signal transduction inhibitor: • Administer antienoplastics: • Monoclonal antibody targeted therapy: • Transfusion if hemolytic anemia or bleeding: • High protein diet.

31. SURGICAL MANAGEMENT: 1. For acute myelogenous leukemia: Autologous or Allogeneic hematopoietic stem cell transplant. 2. For acute lymphocytic leukemia: Allogeneic hematopoietic stem cell transplant.

32. 3. For chronic myelogenous leukemia: Hematopoietic cell transplant, alpha interferon, leukapheresis. 4. For chronic lymphocytic leukemia: Spleenoctomy, allogeneic hematopoietic stem cell transplant

33. NURSING INTERVENTION • Monitor for bleeding—platelet count may be decreased. • Monitor for infection—patients have increased susceptibility to infection. • Monitor pain control. • Small, frequent meals.

34. Cont… • Teach patients about infection control: • Avoid others with infection. • Report signs of infection, sore throat, fevers, etc. • Explain to the patient: • Use an electric razor. • Use soft toothbrush. • Watch for bleeding or bruising

35. 8:Hodgkin disease & Non Hodgkin lymphoma lymphoma A malignant neoplasm originating in the bone marrow and lymphatic system Involves the lymph nodes or other lymphoid organs as the spleen. Characterized by proliferation of lymphocytes

36. Classification Hodgkin's Lymphoma Non - Hodgkin's Lymphoma

37. Etiology Oncoviruses: Increased risk with: Immune deficiency (HIV) Immunosuppressant Associated with auto - immune disorders exposure to Epstein-Barr virus Occupational pesticides, dyes, benzene Genetic predisposition

38. Hodgkin's Lymphoma: 15% of adult lymphomas Age group most affected: 15 - 35 or >50 years Affects males twice as much as females Proliferation of giant multi-nucleated cells in the lymph nodes: Reed-Sternberg cells

39. Pathophysiology Hyperplasia of monocytes and macrophages (lymphoid stem cell) Reed-Sternberg cells originate in a single lymph node Spread via lymphatics to adjacent lymph nodes then to lungs, liver, spleen, CNS If originates above diaphragm confined more to nodes If below diaphragm ↑ spread to liver

40. Clinical Manifestations Presence of non-tender hard swollen lymph node or nodes: cervical (most often first), axillary, inguinal Fever, fatigue, weakness, weight loss Tachycardia, chills, night sweats Cough, dyspnea, dysphagia, stridor Hepatomegaly Splenomegaly

41. Complications Anaemia: ↓ RBC production (pallor/ fatigue) ↑ RBC destruction (pruritis/ jaundice) Pressure signs from advancing tumour : spinal cord compression (paralysis) renal compression (urinary retention) bowel compression (constipation)

42. Diagnosis Biopsy and histology of a lymph node (often cervical): Shows proliferation of giant multi-nucleated Reed- Sternberg cells Staging of disease progression to determine management

43. Staging Stage 1: a single node or extra-nodal site Stage 2: two or more nodes or sites on the same side of the diaphragm Stage 3: involvement on both sides of diaphragm Stage 4: diffused throughout the body

44. Management Treatment is according to the staging Treatment aims at CURE Stage 1 and 2: *Radiotherapy to the affected areas 4 – 6 weeks (sometimes with chemotherapy if thought resistant) Stage 3 and 4: Chemotherapy (and possibly radiotherapy) (poorer prognosis)

45. Non-Hodgkin’s Lymphoma A malignant disorder of the lymphocytes: Mainly the B-cells Spreads via the blood circulation ( Hodgkins spreads via lymphatics) Typically not diagnosed early: May be already stage 3 or 4 at diagnosis Early metastases to CNS.

46. Clinical Manifestations Swollen lymph glands A mass in mediastinum or abdomen Pressure symptoms: Chest tightness, spinal cord compression, intra- abdominal pressure Fatigue and weakness Pyrexia, night sweats Weight loss: >10% body weight

47. Diagnosis Lymph node biopsy: malignant proliferation of B- cell, T-cell, non-B/ non-T Bone marrow biopsy Xray , CT scan, MRI to detect metastases

48. Management Systemic chemotherapy (as well as radiation to affected lymph nodes): Induction phase → Remission Sanctuary phase: (intra- thecal chemotherapy as may have spread to CNS) Maintenance therapy for 2 years Prognosis Poorer prognosis than Hodgkins Lymphoma as often detected late

49. Nursing Considerations Care of patient undergoing chemotherapy Care of patient undergoing radiotherapy Monitoring of blood cell count (CBC) Patient education in relation to lowered immunity Patient and family emotional and psychological support Education for control of symptoms

50. 9:Autoimmune and thrombotic Thrombocytopenic purpura An autoimmune disorder in which antibodies are developed to the patient’s own platelets. Antibodies attach to the platelets and macrophages within the spleen. The destroys the platelets within the spleen. It is typically more common in women and becomes chronic in adults who are in early to mid-adulthood.

51. PROGNOSIS Problems for the patient are most likely the result of bleeding due to inadequate platelets. Prednisone can control the majority of cases.

52. SIGNS AND SYMPTOMS • Bleeding in mucous membranes or skin due to low platelet count: • epistaxis • oral bleeding • menorrhagia (heavy menstrual bleeding) • purpura • petechiae

53. INTERPRETING TEST RESULTS • Thrombocytopenia—low platelet count. • Mild anemia—usually secondary to bleeding. • Prothrombin time ( PT )normal. •  Partial thromboplastin   time  PTT normal.

54. TREATMENT The use of prednisone in patients with Immune thrombocytopenia ITP is to decrease the body’s action on the antibody-tagged platelets. Initially, the use of prednisone will also help to enhance vascular stability. High-dose therapy needs to be tapered down. Most patients will be on long-term maintenance doses of prednisone.

55. Cont…. Splenectomy provides complete or partial remission. • Prednisone—bleeding will stop even before platelet count begins to rise. • High-dose IV immunoglobulin. • Immunosuppressive therapy • Stem cell transplantation.

56. NURSING INTERVENTIONS • Monitor vital signs for changes. • Monitor for signs of bleeding or bruising. • Decrease chance of bleeding: • Use soft toothbrushes, no flossing, only electric razors. • Protect from potential infection, sick visitors, etc. • Encourage patient to discuss feelings about illness.

57. Disseminated intravascular coagulation  ( DIC ) Disseminated intravascular coagulation  ( DIC ) is a rare but serious condition that causes abnormal blood clotting throughout the body's blood vessels. It is caused by another disease or condition, such as an infection or injury, that makes the body's normal blood clotting process become overactive

58. 10:Disseminated Intravascular Coagulation (DIC) Blood coagulates through the entire body within the vascular compartment. This depletes platelets and the body’s ability to coagulate, resulting in an increased risk of hemorrhage. It occurs as a complication of some other condition. The coagulation sequence is activated causing many micro thrombi to develop throughout the body.

59. The clots that form are the result of coagulation proteins and platelets, resulting in the risk of bleeding or severe hemorrhage. It is often due to obstetric complications, post trauma, sepsis, cancer, or shock.

60. Clinical manifestationcid • Unexpected bleeding—oozing from puncture sites (venipuncture, IVs, surgical wounds) • Petechiae as clotting factors are lost • Purpura as clotting factors are lost • Severe hemorrhage as clotting factors are lost • Uncontrolled postpartum bleeding • Tissue hypoxia from microemboli

61. • Hemolytic anemia, as cells are destroyed trying to pass through partially blocked vessels

62. INTERPRETING TEST RESULTS • PT prolonged. • PTT normal or prolonged. • Platelet count low—thrombocytopenia. • Fibrin degradation products elevated: • D- dimer may be elevated.

63. TREATMENT Treatment needs to decrease coagulation ability (to prevent further clot development) and replace clotting components (to prevent further bleeding). Other interventions may be necessary depending on the locations of clot development and any compromise of body system function due to clot formation.

64. Cont.. • Transfusion: • Packed RBC • Fresh frozen plasma—replaces coagulation factor deficiency • Platelets—replaces needed cells. • Cryoprecipitate—replaces fibrinogen. • Administer anticoagulant drugs to decrease coagulation; not done in all patients: • heparin • Bed rest.

65. NURSING INTERVENTION • Monitor for bleeding from obvious sites (wounds, suture lines)and occult sites (GI, urine). • Avoid cleaning clots from exposed areas—may start bleeding from the site and not have sufficient clotting factors to stop. • Explain to the patient: • Avoid situations that might cause bleeding—use electric razor, soft toothbrush, don’t floss between teeth.

66. Thank you