Hematology aplasticanemia-120108092936-phpapp02

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Hematology aplasticanemia-120108092936-phpapp02

  1. 1. Aplastic Anemia Dr. Kalpana Malla MD Pediatrics Manipal Teaching HospitalDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
  2. 2. PANCYTOPENIA:• Simultaneous presence of anaemia, leukopenia, thrombocytopenia• Causes: aplastic anemia Subleukemic leukemia cytotoxic drugs radiotherapy bone marrow infiltration hypersplenism megaloblastosis SLE
  3. 3. APLASTIC ANEMIA:• Failure of two or more cell lines• Anaemia, leukopenia, thrombocytop enia + Hypoplasia or aplasia of the marrow
  4. 4. Pathology:• Reduction in the amount of haemopoietic tissue- inability to produce mature cells for discharge into the bloodstream• Patchy areas of normo/hypercellularity betn areas of hypocellularity
  5. 5. CLASSIFICATION:• Idiopathic• Secondary: idiosyncratic drug reaction chemical exposure infectious hepatitis paroxysmal nocturnal haemoglobinuria• Constitutional
  6. 6. CLASSIFICATIONCONSTITUTIONAL/CONGENITAL• Diamond-Blackfan syndrome• Shwachmann-Diamond syndrome• Fanconi anemia• Dyskeratosis Congenita• TAR (thrombocytopenia with absent radii)• Amegakaryocytic thrombocytopenia
  7. 7. FANCONI ANEMIA• Familial• AR• M:F=1.3:1• Onset in 1st decade of life• Diminished capacity for DNA repair and increased random chromosome breakage during mitosis
  8. 8. C/F:• Facies – microphthalmia,depressed nasal bridge,epicanthic fold,micrognathia• Hyperpigmentation, café-au-lait spots• Absent / hypoplastic thumb• Skeletal and renal lesions• Short stature,• Microcephaly , subnormal intelligence• Hypogonadism,ano malies of urinary tract• Predisposition to leukemias• Poor prognosis
  9. 9. DYSKERATOSIS CONGENITA• X-linked, AR, AD• M:F= 4.3:1• Hyperpigmentation• Nail dystrophy, early loss of teeth• Leukoplakia• Ocular abnormalities: cataract etc• Short stature but No skeletal/renal lesions (diff from FA)
  10. 10. DIAMOND BLACKFAN SYNDROME• Congenital pure red cell aplasia• AD, AR, Sporadic• Familial in 15 %• 90 % diagnosed in 1st year of life• Intrinsic defect in RBC, early apoptosis• Macrocytic anemia, reticulocytopenia, absence of RBC precursors in an otherwise normocellular bone marrow
  11. 11. DIAMOND BLACKFAN SYNDROME• Eye - Wide set eyes, blue sclera,glaucoma,epicanthic fold,cataract,strabismus• Thick upper,cleft lip palate in some cases• lip,intelligent expression• Upperlimb anomalies – flattening of thenar eminence, Triphalangeal thumb
  12. 12. C/F:• Profound anemia at 2-6 months of age• Short stature• Renal anomalies and hypogonadism maybe present
  13. 13. ACQUIRED APLASTIC ANEMIA - CAUSES• Radiation • Immune diseases:• Drugs and chemicals - eosinophilic fascitis - chemotherapy - thymoma - benzene • Pregnancy - chloramphenicol • PNH - antiepileptics • Marrow replacement:• Viruses: - leukemia - CMV - myelofibrosis - EBV - myelodysplasia - Hep B, C,D - HIV
  14. 14. PATHOPHYSIOLOGY• Direct destruction of haemopoietic progenitors• Disruption of marrow micro-environment• Immune –mediated suppression of marrow elements  Cytotoxic T cells in blood and marrow release gamma IFN and TNF -> inhibit early and late progenitor cells
  15. 15. Pathology:• Hallmark: peripheral pancytopenia with hypoplastic/ aplastic bone marrow
  16. 16. CLINICAL FEATURESRBC (anemia)• Progressive and persistent pallor• Anemia related symptomsWBC (Leucopenia/neutropenia)• Prone to infections - Pyodermas, OM, pneumonia, UTI, GI infections, sepsisPlatelets (Thrombocytopenia)• Petechiae, purpura, ecchymoses• Hematemesis, hematuria, epistaxis, gingival bleed• IC bleed- headache, irritability, drowsiness, coma
  17. 17. NO HEPATOMEGALY NO SPLENOMEGALY NO LYMPHADENOPATHYFailure of entire RES. No extramedullary hematopoesis
  18. 18. Blood picture:• Anemia-normocytic, normochromic• Leukopenia (neutropenia)• Relative lymphocytosis• Thrombocytopenia• Absolute reticulocyte count low• Mild to moderate anisopoikilocytosis
  19. 19. Other investigations• BM : dry aspirate, hypocellular with fat (>70% yellow marrow)
  20. 20. SEVERITYSEVERE APLASTIC ANEMIA• Granulocyte count <500/cu.mm• Platelet count <20,000/cu.mm• Reticulocytes <1%• BM < 25% of hematopoeitic cellsVERY SEVERE/ EXTREME APLASTIC ANEMIA• Above + Granulocyte count <200/cu.mm
  21. 21. DIFFERENTIAL DIAGNOSIS• ITP• LEUKEMIA• MYELOID METAPLASIA
  22. 22. Management:• Identification and elimination of underlying cause• Supportive therapy:1. Red cell transfusion for anemia2. Prevention and treatment of haemorrhage3. Prevention and treatment of infection
  23. 23. SUPPORTIVE CARE• Prophylactic antibiotics• If infection: cephalosporins + aminoglycosides + metronidazole• Antifungals: amphotericin B, fluconazole (if fever >10 days despite antibiotics)
  24. 24. THERAPYDEFINITIVE• BONE MARROW TRANSPLANTATION• IMMUNOSUPPRESSION
  25. 25. BONE MARROW TRANSPLANTATION• Treatment of choice• HLA matched donor. Usually siblings• Long term survival rates: 60-70%• Donor stem cells > 4 X 108 cells/kg
  26. 26. IMMUNOSUPPRESSION• Antithymocyte globulin (ATG)• Antilymphocyte glubulin (ALG)• Cyclosporin• Intensive immunosupression : cyclophosphamide• Corticosteroids
  27. 27. ATG administration:• IV administration of Ig preparations containing antibody to human thymocytes• Improvement in haematological indices in one-half of subjects• Anaphylaxis
  28. 28. ATG AND ALG• Dose : 40 mg/kg/day X 4 days• Hematologic response rate 45%• Survival rate 60%• Side effects: serum sickness
  29. 29. ANDROGENS• No longer have primary role• Increase erythopoietin producion• Stimulate erythroid stem cells• Increase Hb levels in normal males methyl testosterone, testesterone enanthate, oxymetholone, danazolOral dose : 2-5mg/kg/day, IM dose: 1-2mg/kg/wkSide effects: cholestatic jaundice, masculinization
  30. 30. Corticosteroids:• High dose steroids combined with anabolic agents• Uncertain benefits• Side-effects
  31. 31. HAEMATOPOIETIC GROWTH FACTORS• GM-CSF• G-CSF• IL-3• IL-1• IL-6
  32. 32. Thank youDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]

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