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Aplastic anemia 2011
Aplastic anemia is a bone marrow failure syndrome characterized by pancytopenia and a hypocellular bone marrow. It is diagnosed through a bone marrow biopsy showing fewer than 25% of normal hematopoietic cells. The causes of aplastic anemia include exposure to toxins, certain drugs, viruses, and an immune-mediated process destroying hematopoietic stem cells in the bone marrow. Treatment involves immunosuppressive therapy with antithymocyte globulin and cyclosporine, or allogeneic bone marrow transplantation for severe cases. Long term management focuses on supportive care through blood product transfusions and monitoring for complications.
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Aplastic anemia 2011
- 1. Aplastic anemia • approach – –
- 2. Diagnosis • Pancytopenia • Exclude • Hypocellular marrow – Acute leukemia – Aspiration – Myelodysplastic – Biopsy syndrome – Paroxysmal nocturnal hemoglobinuria
- 3.
- 5. aplastic anemia • exposure to – nonbottled water – certain animals (ducks and geese) – animal fertilizer – pesticides
- 6. aplastic anemia historical studies • Drugs • Chemicals • Pregnancy • Eosinophilic fasciitis • Hepatitis - seronegative
- 7. • occupational exposure • • • bone marrow transplantation)
- 8. Proper Investigations • CBC, blood smear • Bone marrow aspiration and biopsy • Chromosome study (in elderly patients) • PNH work up • Hepatitis viruses • Complete blood typing
- 9. Severe Aplastic Anemia Peripheralblood • Granulocyte < 500/mm3 • Platelet < 20,000/mm3 • Reticulocyte count < 1% Bone marrow • Cellularity < 25% • Cellularity 25-50% hematopoietic cells < 30%
- 10. Aplastic anemia isan immune process • What else could be so effective and selective?
- 11. aplastic anemia immune process • clinical – Autologous recovery after stem cell transplantation – Failed syngeneic transplantation – Response to immunosuppresion • lab – Lymphocyte co-culture
- 12. Cell ,cytokine mechanism • Th1 lymphocytes
- 13. Proximal events inimmune- mediated marrow failure
- 14. Apoptosis • apoptosis stem cell • upregulation gene apoptosis • cell expose gamma interferon
- 15. mechanism AA PNH • “Escape” from immune process GPI-linked membrane proteins
- 16. MDS presentation respond to treatment AA mutation • Trisomy 8 • Upregulation anti-apoptosis genes
- 17. Management of SevereAA • 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
- 18. Results of BMTin AA (from NMDP, USA)
- 19. BMT AA 2 • Chronic GVHD – Irradiation – Heavy cell dose – Increasing age • Rejection – Heavy previous transfusion – Low cell dose
- 20. long tem surivival AA ATG CsA 3 11 • 3 65-70% • 11 39%
- 21. Risk relapse ATG CsA • 30 – 40 % • prolonged CsA (> 6 months)
- 22. Long term CsAdependence ATG CsA • 26-62%
- 23. role additional G-CSF ATG CsA • neutrophil trilineage hematological recovery survival
- 24. ATG •2 • response 50-60% • relapse
- 25. Management of non-severe AA • Anabolic hormones – Anticipated response > 3 • Cyclosporin alone
- 26. supportive treatment • AA • • Decreased quality of life • Loss of work opportunity • High expense of treatment
- 27. Transfusion guidelines • BMT • leukodepleted blood products • Platelet transfusion Prophylactic vs. Therapeutics