Antithymocyte globulin and @entity205 for severe XXXX : association between hematologic response and long-term outcome.

@entity1 @entity1667 @entity2150 @entity1503 @entity205 @entity3043 @entity288
CONTEXT: In most @entity1 , @entity2150 results from T-cell-mediated immune destruction of bone marrow. @entity2150 can be effectively treated by stem cell transplantation or immunosuppression. OBJECTIVE: To assess long-term outcomes after immunosuppressive therapy. DESIGN, SETTING, AND @entity1 : Cohort of 122 @entity1 (31 were < or =18 years and 91 were >18 years) with severe @entity2150 , as determined by bone marrow cellularity and blood cell count criteria, were enrolled in a single-arm interventional research protocol from 1991 to 1998 at a federal government research hospital. INTERVENTIONS: A dose of 40 mg/kg per day of antithymocyte globulin administered for 4 days, 10 to 12 mg/kg per day of @entity205 for 6 months (adjusted for blood levels), and a short course of @entity288 (1 mg/d of @entity1667 for about 2 weeks). MAIN OUTCOME MEASURES: Survival, improvement of @entity3043 and transfusion-independence, relapse, and evolution to other @entity1503 . RESULTS: Response rates were 60% at 3 months after initiation of treatment, 61% at 6 months, and 58% at 1 year. The blood cell counts of @entity1 who responded no longer satisfied severity criteria and they were transfusion-independent. Overall actuarial survival at 7 years was 55%. Survival was associated with early satisfaction of response criteria (86% vs 40% at 5 years; P<.001) and by blood counts at 3 months (reticulocyte count or platelet count of >50 x 10(3)/ microL predicted survival at 5 years of 90% [64/71] vs 42% [12/34] for @entity1 with less robust recovery [P<.001 by log-rank test]). There were no deaths among responders more than 3 years after treatment. Relapse was common, but severe @entity3043 usually did not recur. Relapse did not influence survival. Thirteen @entity1 showed evolution to other @entity1503 , including monosomy 7. CONCLUSIONS: Approximately half of @entity1 with severe @entity2150 treated with antithymocyte globulin and @entity205 have durable recovery and excellent long-term survival. These outcomes were related to the quality of hematologic recovery.
