Bezirgiannidou Zoe, Stamatiou Iliana, Theodoridis Theodoros M, Kontekaki Eftychia, Panagiotopoulos Emmanouil, Misidou Christina, Vrachiolias George, Malkots Bouse, Papoutselis Menelaos, Kotsianidis Ioannis, Spanoudakis Emmanouil, Liapis Konstantinos
Department of Hematology, University Hospital of Alexandroupolis Democritus University of Thrace Alexandroupolis, 681 00, Alexandroupolis, Greece.
Wireless Communications and Information Processing (WCIP) Group, Electrical & Computer Engineering Dept, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Ann Hematol. 2025 Aug 28. doi: 10.1007/s00277-025-06585-7.
This report describes the first successful administration of ravulizumab, a C5 complement inhibitor, in the treatment of life-threatening intravascular hemolysis (IVH) caused by delayed hemolytic transfusion reaction (DHTR) in a 22-year-old woman. The patient developed acute IVH with severe anemia and hemodynamic instability seven days after receiving a blood transfusion for posthemorrhagic anemia following a missed abortion. Laboratory investigations revealed anti-e and anti-Jka alloantibodies consistent with DHTR. Despite treatment, her hemoglobin level declined further, raising concerns for hyperhemolytic syndrome. After the administration of ravulizumab, her condition improved rapidly, and she was discharged with stable hemoglobin levels. Within three weeks there was full hematologic and biochemical recovery. This case demonstrates the therapeutic potential of ravulizumab in the management of severe complement-mediated hemolysis due to DHTR, and highlights the need for further research on complement inhibitors in similar conditions.
本报告描述了在一名22岁女性中首次成功使用C5补体抑制剂ravulizumab治疗由延迟性溶血性输血反应（DHTR）引起的危及生命的血管内溶血（IVH）。该患者在因稽留流产后出血性贫血接受输血7天后出现急性IVH，伴有严重贫血和血流动力学不稳定。实验室检查发现与DHTR一致的抗 - e和抗 - Jka同种抗体。尽管进行了治疗，她的血红蛋白水平仍进一步下降，引发了对高溶血性综合征的担忧。给予ravulizumab后，她的病情迅速改善，出院时血红蛋白水平稳定。三周内血液学和生化指标完全恢复。该病例证明了ravulizumab在治疗由DHTR引起的严重补体介导的溶血方面的治疗潜力，并强调了在类似情况下对补体抑制剂进行进一步研究的必要性。
