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Gérard Socié, MD PhD Hospital Saint Louis

AIH. Gérard Socié, MD PhD Hospital Saint Louis. SAA Hypo cellular marrow (<30%) & at least 2/3 criteria: ANC <0.5x10 9 /L Platelets <20x10 9 /L Reticulocytes <20x10 9 /L v.SAA ANC <0.2x10 9 /L moderate AA not fulfilling criteria of SAA ANC >0.5x10 9 /L. Hemophagocytosis

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Gérard Socié, MD PhD Hospital Saint Louis

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  1. AIH Gérard Socié, MD PhD Hospital Saint Louis

  2. SAA Hypo cellular marrow (<30%) & at least 2/3 criteria: ANC <0.5x109/L Platelets <20x109/L Reticulocytes <20x109/L v.SAA ANC <0.2x109/L moderate AA not fulfilling criteria of SAA ANC >0.5x109/L

  3. Hemophagocytosis by an immature myeloid cell Dysplastic changes in the few scattered hematopoietic cells MDS or SAA? Fibrosis Splenomegaly Blasts Dysplastic megakaryocyte myeloid Cytogenetics? PNH? Megaloblastic proerythroblast, coarse chromatin, multiple nucleoli cytoplasmic budding Dysplastic changes in the myeloid series

  4. Does this guideline hold true?

  5. HSCT HLA-id. Sibling D Ades et al. Blood. 2004;103: 2490-2497

  6. Better outcome - early BMT - few transfusions - age - GvHD prophylaxis CSA+MTX 5 year Survival <1985: 51% + 4 n= 741 1985-94: 63% + 2 n=1384 1995-04: 69% + 2 n=2044

  7. Cy - TAI Cy - ATG Time from transplant (year)

  8. Time period N Survival 5 years 10 years <1980 577 54±4% 46±4% 1980-1990 413 67±5% 61±5% 1990-2000 1114 73±3% 69±4% >2000 160 77±8% - Immunosuppression

  9. Immunosuppressive treatment Best results with ATG + CSA Bacigalupo A. Blood 2000; 95(6):1931-1934.

  10. Socié G. NEJM. 1993; (329). 1152-1157 Bacigalupo A, Blood 2000; (95). 1931-1934 Frickhofen N. Blood. 2003 (101). 1236-1242

  11. Socié G. et al., Submitted 2006

  12. UD transplant SFGM-TC > 1998 < 1998

  13. SFGM-TC • In multivariate analysis: survival • HLA allelic matching (P<.01) • younger age of (<17 years, P<.0001). • Survival reached 78%±11% at 5-year for the younger patients HLA-matched.

  14. Identical sibling As early as possible Bone marrow Cy + ATG CSA +MTX MUD after failed IS 1 - 2 courses? Conditioning? GvHD Prophylaxis?

  15. IS good results with H-ATG + CSA G-CSF still need randomized trial no upper age limit refractory patients? other immunosuppressant? MMF ineffective Campath? Sirolimus? Anti-CD25? Rituximab? BMT good results with Id Sibling BMT MUD BMT (2nd line) Older (Age old??) Alternative donors? Cord?

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