Ma Kim, Caplan Stephen
Department of Medicine, Division of Hematology, Jewish General Hospital, 3755 Chemin de la Côte-Sainte-Catherine, Room E-725, Montreal, QC, Canada H3T 1E2.
Case Rep Hematol. 2016;2016:9181698. doi: 10.1155/2016/9181698. Epub 2016 Mar 22.
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.
温抗体型自身免疫性溶血性贫血(wAIHA)是自身免疫性溶血性贫血最常见的形式,一线治疗使用皮质类固醇的缓解率为60%-80%。非典型wAIHA以及IgG加补体介导的疾病治疗失败率更高且复发率更高。我们报告1例继发于华氏巨球蛋白血症的严重wAIHA病例,该患者出现危及生命的血管内溶血,对泼尼松、利妥昔单抗、脾切除术和血浆置换均无效。对该经过大量预处理的患者进行四周的依库珠单抗治疗后,血红蛋白持续升高且不再需要输血,提示补体抑制在难治性wAIHA中发挥作用。