Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy
نویسندگان
چکیده
Warm autoimmune hemolytic anemia (wAIHA) is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60-80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysis refractory to prednisone, rituximab, splenectomy, and plasmapheresis. A four-week treatment of eculizumab in this heavily pretreated patient resulted in a sustained increase in hemoglobin and transfusion independence, suggesting a role for complement inhibition in refractory wAIHA.
منابع مشابه
Successful Treatment of Refractory Autoimmune Hemolytic Anemia (AIHA) in a Child, Based on Iranian Traditional Medicine: A Case Report
Autoimmune hemolytic anemia (AIHA) is a heterogeneous and relatively unknown disease caused by premature immune destruction of red blood cells. While its occurrence is uncommon among children, it is sometimes severe and resistant to treatment. The warm-reactive type contains 70% to 80% of all cases, in which the first-line treatment is considered to be a steroid. Moreover, splenectomy, rituxima...
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A 58-year-old man with warm-antibody-mediated autoimmune hemolytic anemia (AIHA) refractory to prednisolone, azathioprine, splenectomy, rituximab and combination chemotherapy, and with unacceptably high transfusion requirement, was treated with alemtuzumab. After a cumulative dose of 883 mg of alemtuzumab, the AIHA remitted completely, with normalization of hemoglobin and transfusion-independen...
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Polygenic IgG autoantibodies are implicated in majority of the cases of warm autoimmune hemolytic anemia (WAIHA). In some of these cases, complement (C3) proteins accompany the IgG antibodies. WAIHA mediated by C3 alone is relatively rare. We present an interesting case of WAIHA with a direct antiglobulin test (DAT) positive for C3 but negative for IgG in a 79-year-old woman and perform an anal...
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BACKGROUND Autoimmune hemolytic anemia is rare in children. First-line therapies for this disease consist of corticosteroids and intravenously administered immunoglobulin that are effective in most patients. However, a small proportion of cases (5 to 10%) is refractory to these therapies and may represent a medical emergency, especially when hemolysis is due to warm immunoglobulin M. Recently, ...
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ورودعنوان ژورنال:
دوره 2016 شماره
صفحات -
تاریخ انتشار 2016