Koppel Ahrin, Lim Stephen, Osby Melanie, Garratty George, Goldfinger Dennis
Department of Medicine, Division of Hematology-Oncology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Transfusion. 2007 Oct;47(10):1902-4. doi: 10.1111/j.1537-2995.2007.01414.x.
Paroxysmal cold hemoglobinuria (PCH) is a rare autoimmune hemolytic anemia (AIHA) attributed to a biphasic hemolysin known as the Donath-Landsteiner (DL) antibody. It is most commonly encountered as an acute transient AIHA after a viral infection in children; the disease resolves after cessation of the infection. The rarest form of PCH is a chronic form in adults that is not (nowadays) associated with infection and is not responsive to conventional therapies. Rituximab has been found to be effective therapy in other forms of AIHA, such as cold agglutinin syndrome, that are refractory to conventional therapies. We describe a case of PCH refractory to steroids that responded to rituximab therapy on two separate occasions.
A 64-year-old woman with fatigue was found to be profoundly anemic with laboratory findings consistent with AIHA. She was admitted for the workup and management of her disease after she failed to respond to a course of oral steroids. Laboratory evaluation demonstrated a positive DL test suggesting PCH. She was given a course of rituximab that resulted in normalization of her hemoglobin concentration. She presented 9 months later with recurrent hemolysis. She was given another course of rituximab that again resulted in termination of hemolysis. The patient remained in remission since her last dose of rituximab 19 months previously.
To our knowledge, this is the first report of an adult case of refractory PCH successfully treated with rituximab.
阵发性冷血红蛋白尿（PCH）是一种罕见的自身免疫性溶血性贫血（AIHA），由一种称为多纳-兰斯泰纳（DL）抗体的双相溶血素引起。它最常见于儿童病毒感染后的急性短暂性AIHA；感染停止后疾病可缓解。PCH最罕见的形式是成人慢性形式，（如今）与感染无关，对传统疗法无反应。利妥昔单抗已被发现对其他形式的AIHA有效，如对传统疗法难治的冷凝集素综合征。我们描述了一例对类固醇难治的PCH病例，该病例在两次单独的情况下对利妥昔单抗治疗有反应。
一名64岁疲劳女性被发现严重贫血，实验室检查结果与AIHA一致。在口服类固醇治疗疗程无效后，她因疾病的检查和管理入院。实验室评估显示DL试验阳性，提示PCH。她接受了一个疗程的利妥昔单抗治疗，血红蛋白浓度恢复正常。9个月后，她因溶血复发再次就诊。她又接受了一个疗程的利妥昔单抗治疗，溶血再次终止。自19个月前最后一剂利妥昔单抗以来，患者一直处于缓解状态。
据我们所知，这是首例用利妥昔单抗成功治疗的成人难治性PCH病例报告。
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