2016
DOI: 10.1155/2016/9181698
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Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy

Abstract: 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 he… Show more

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Cited by 15 publications
(13 citation statements)
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“…20 The utility of anticomplement therapy, such as eculizumab, for warm AIHA is unknown, although its use in cold agglutinin disease and in isolated severe cases of refractory warm AIHA with a DAT positive for both IgG and complement have been reported. 21,22 Our patient's DAT was negative for complement, similar to a majority of the prior case reports (although a confounding factor is the concomitant anti Wr b or anti-dl). As such, we would not predict a therapeutic role for anticomplement drugs, such as eculizumab, for such patients.…”
Section: Discussionsupporting
confidence: 85%
“…20 The utility of anticomplement therapy, such as eculizumab, for warm AIHA is unknown, although its use in cold agglutinin disease and in isolated severe cases of refractory warm AIHA with a DAT positive for both IgG and complement have been reported. 21,22 Our patient's DAT was negative for complement, similar to a majority of the prior case reports (although a confounding factor is the concomitant anti Wr b or anti-dl). As such, we would not predict a therapeutic role for anticomplement drugs, such as eculizumab, for such patients.…”
Section: Discussionsupporting
confidence: 85%
“…Both show promise in severe refractory autoimmune diseases including cytopenias (28-31), but have yet to be trialed in PID specifically. Finally, the terminal complement inhibitor eculizumab (anti-C5) has been utilized to rescue a patient from fatal complications related to treatment-refractory AIHA (32). Since it acts distal to the B cell in autoantibody mediated diseases, it could in theory be applied in combination with B cell depleting therapies to more completely control disease.…”
Section: Treatment Of Autoimmune Cytopenias In Primary Immunodeficmentioning
confidence: 99%
“…There are however two published reports of clinical efficacy of eculizumab in wAIHA, in IgM-mediated wAIHA secondary to Churg-Strauss syndrome,13 and in refractory IgG-mediated wAIHA secondary to Waldenstrom macroglobulinaemia 14. Both cases had evidence of complement activation, with DAT positivity for C3d.…”
Section: Discussionmentioning
confidence: 98%