Department of Hematology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano City, Nagano, 380-8582, Japan.
Department of Rheumatology, Minami Nagano Medical Center, Shinonoi General Hospital, Nagano, Japan.
Int J Hematol. 2024 Dec;120(6):735-742. doi: 10.1007/s12185-024-03851-8. Epub 2024 Sep 20.
Paroxysmal cold hemoglobinuria (PCH) is a form of cold autoimmune hemolytic anemia characterized by the presence of the Donath-Landsteiner antibody, which triggers complement-mediated intravascular hemolysis when the body temperature changes from cold to warm. PCH occurs primarily in children as a rare, self-limiting disease following viral infections. In contrast, adult-onset PCH is very rare and associated with a diverse range of underlying conditions, which complicates its management and treatment. We describe a case of adult-onset PCH following COVID-19, effectively managed with a single dose of sutimlimab, a selective classical complement pathway inhibitor. This intervention was performed during a life-threatening hemolytic crisis, at a time requiring swift decision-making when specific tests to differentiate from other hemolytic anemias were not readily available. This case illustrates the potential of using a single dose of sutimlimab to manage life-threatening hemolytic crises in PCH, highlighting the significance of inhibiting the classical complement pathway.
阵发性冷性血红蛋白尿症(PCH)是一种冷自身免疫性溶血性贫血,其特征是存在 Donath-Landsteiner 抗体,当体温从冷变热时,该抗体触发补体介导的血管内溶血。PCH 主要发生在儿童中,是一种罕见的自限性疾病,继发于病毒感染。相比之下,成人发病的 PCH 非常罕见,与多种潜在疾病相关,这使得其管理和治疗变得复杂。我们描述了一例 COVID-19 后成人发病的 PCH,通过单次使用 sutimlimab(一种选择性经典补体途径抑制剂)有效治疗。该干预措施在危及生命的溶血性危象期间进行,当时需要快速决策,而特定的测试来区分其他溶血性贫血并不容易获得。该病例说明了使用单次剂量 sutimlimab 来管理 PCH 危及生命的溶血性危机的潜力,突出了抑制经典补体途径的重要性。