Aplastic anemia 2011

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Slide สอน R med เรื่อง aplastic anemia and bone marrow failure

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Aplastic anemia 2011

  1. 1. Aplastic anemia• approach – –
  2. 2. Diagnosis• Pancytopenia • Exclude• Hypocellular marrow – Acute leukemia – Aspiration – Myelodysplastic – Biopsy syndrome – Paroxysmal nocturnal hemoglobinuria
  3. 3. Bone marrow pathology
  4. 4. aplastic anemia• exposure to – nonbottled water – certain animals (ducks and geese) – animal fertilizer – pesticides
  5. 5. aplastic anemia historical studies• Drugs• Chemicals• Pregnancy• Eosinophilic fasciitis• Hepatitis - seronegative
  6. 6. • occupational exposure••• bone marrow transplantation)
  7. 7. Proper Investigations• CBC, blood smear• Bone marrow aspiration and biopsy• Chromosome study (in elderly patients)• PNH work up• Hepatitis viruses• Complete blood typing
  8. 8. Severe Aplastic AnemiaPeripheral blood• Granulocyte < 500/mm3• Platelet < 20,000/mm3• Reticulocyte count < 1%Bone marrow• Cellularity < 25%• Cellularity 25-50% hematopoietic cells < 30%
  9. 9. Aplastic anemia is an immune process• What else could be so effective and selective?
  10. 10. aplastic anemia immune process• clinical – Autologous recovery after stem cell transplantation – Failed syngeneic transplantation – Response to immunosuppresion• lab – Lymphocyte co-culture
  11. 11. Cell , cytokine mechanism• Th1 lymphocytes
  12. 12. Proximal events in immune- mediated marrow failure
  13. 13. Apoptosis• apoptosis stem cell• upregulation gene apoptosis• cell expose gamma interferon
  14. 14. mechanism AA PNH• “Escape” from immune process GPI-linked membrane proteins
  15. 15. MDS presentation respond to treatment AA mutation• Trisomy 8• Upregulation anti-apoptosis genes
  16. 16. Management of Severe AA• BMT or ATG or > 70%• Antithymocyte globulin – Better results when combined with cyclosporin – Anticipated response > 3• Allogeneic bone marrow transplantation, esp. in – Young patient – Patient with very severe AA
  17. 17. Results of BMT in AA (from NMDP, USA)
  18. 18. BMT AA 2• Chronic GVHD – Irradiation – Heavy cell dose – Increasing age• Rejection – Heavy previous transfusion – Low cell dose
  19. 19. long tem surivival AA ATG CsA 3 11• 3 65-70%• 11 39%
  20. 20. Risk relapse ATG CsA• 30 – 40 %• prolonged CsA (> 6 months)
  21. 21. Long term CsA dependence ATG CsA• 26-62%
  22. 22. role additional G-CSF ATG CsA• neutrophil trilineage hematological recovery survival
  23. 23. ATG•2• response 50-60%• relapse
  24. 24. Management of non-severe AA• Anabolic hormones – Anticipated response > 3• Cyclosporin alone
  25. 25. supportive treatment• AA•• Decreased quality of life• Loss of work opportunity• High expense of treatment
  26. 26. Transfusion guidelines• BMT• leukodepleted blood products• Platelet transfusion Prophylactic vs. Therapeutics

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