CASE REPORT article
Front. Immunol.
Sec. Autoimmune and Autoinflammatory Disorders : Autoimmune Disorders
Volume 16 - 2025 | doi: 10.3389/fimmu.2025.1618807
Severe hemolysis flare of refractory warm hemolytic anemia with positive complement component C3d responsive to Iptacopan with cyclophosphamide and prednisone
Provisionally accepted
- Department of Hematology, THe First Affiliated Hospital, Nanjing Medical University, Nanjing, China
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Warm autoimmune hemolytic anemia (wAIHA) is typified by the autoimmunemediated destruction of erythrocytes. Both wAIHA and Evans syndrome are rare, manifesting a severe clinical course, a high relapse rate, and potentially fatal outcomes.Refractory wAIHA shows poor responsiveness to multiple treatment regimens. In this report, we describe three refractory cases with positive complement component C3d and hemolysis flare. These patients were successfully treated with oral factor B inhibitor Iptacopan in conjunction with cyclophosphamide and prednisone. All three patients showed a rapid increase in Hb levels, a decrease in reticulocyte proportion, and significant reduction in hemolysis manifestations (Lower LDH level and unconjugated bilirubin). These cases provided a potentially effective treatment option for severe hemolysis episodes in refractory wAIHA.
Keywords: autoimmune hemolytic anemia, Evans syndrome, iptacopan, Complement inhibitor, complement component C3d
Received: 27 Apr 2025; Accepted: 04 Aug 2025.
Copyright: © 2025 Zhang, Gong and He. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Guangsheng He, Department of Hematology, THe First Affiliated Hospital, Nanjing Medical University, Nanjing, China
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