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Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
Aplastic anemia
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Aplastic anemia

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  • 1. Aplastic Anemia Dr. Kalpana Malla MD Pediatrics Manipal Teaching HospitalDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
  • 2. PANCYTOPENIA:• Simultaneous presence of anaemia, leukopenia, thrombocytopenia• Causes: aplastic anemia Subleukemic leukemia cytotoxic drugs radiotherapy bone marrow infiltration hypersplenism megaloblastosis SLE
  • 3. APLASTIC ANEMIA:• Failure of two or more cell lines• Anaemia, leukopenia, thrombocytop enia + Hypoplasia or aplasia of the marrow
  • 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. CLASSIFICATION:• Idiopathic• Secondary: idiosyncratic drug reaction chemical exposure infectious hepatitis paroxysmal nocturnal haemoglobinuria• Constitutional
  • 6. CLASSIFICATIONCONSTITUTIONAL/CONGENITAL• Diamond-Blackfan syndrome• Shwachmann-Diamond syndrome• Fanconi anemia• Dyskeratosis Congenita• TAR (thrombocytopenia with absent radii)• Amegakaryocytic thrombocytopenia
  • 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. 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. 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. 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. 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. C/F:• Profound anemia at 2-6 months of age• Short stature• Renal anomalies and hypogonadism maybe present
  • 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. 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. Pathology:• Hallmark: peripheral pancytopenia with hypoplastic/ aplastic bone marrow
  • 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. NO HEPATOMEGALY NO SPLENOMEGALY NO LYMPHADENOPATHYFailure of entire RES. No extramedullary hematopoesis
  • 18. Blood picture:• Anemia-normocytic, normochromic• Leukopenia (neutropenia)• Relative lymphocytosis• Thrombocytopenia• Absolute reticulocyte count low• Mild to moderate anisopoikilocytosis
  • 19. Other investigations• BM : dry aspirate, hypocellular with fat (>70% yellow marrow)
  • 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. DIFFERENTIAL DIAGNOSIS• ITP• LEUKEMIA• MYELOID METAPLASIA
  • 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. SUPPORTIVE CARE• Prophylactic antibiotics• If infection: cephalosporins + aminoglycosides + metronidazole• Antifungals: amphotericin B, fluconazole (if fever >10 days despite antibiotics)
  • 24. THERAPYDEFINITIVE• BONE MARROW TRANSPLANTATION• IMMUNOSUPPRESSION
  • 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. IMMUNOSUPPRESSION• Antithymocyte globulin (ATG)• Antilymphocyte glubulin (ALG)• Cyclosporin• Intensive immunosupression : cyclophosphamide• Corticosteroids
  • 27. ATG administration:• IV administration of Ig preparations containing antibody to human thymocytes• Improvement in haematological indices in one-half of subjects• Anaphylaxis
  • 28. ATG AND ALG• Dose : 40 mg/kg/day X 4 days• Hematologic response rate 45%• Survival rate 60%• Side effects: serum sickness
  • 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. Corticosteroids:• High dose steroids combined with anabolic agents• Uncertain benefits• Side-effects
  • 31. HAEMATOPOIETIC GROWTH FACTORS• GM-CSF• G-CSF• IL-3• IL-1• IL-6
  • 32. Thank youDownload more documents and slide shows on The Medical Post [ www.themedicalpost.net ]

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