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地拉罗司治疗改善了造血功能,并使纯红细胞再生障碍性贫血患者获得完全缓解。

Deferasirox treatment improved hematopoiesis and led to complete remission in a patient with pure red cell aplasia.

机构信息

Division of Hematology/Oncology, Department of Internal Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1143, Japan,

出版信息

Int J Hematol. 2013 Dec;98(6):719-22. doi: 10.1007/s12185-013-1455-0. Epub 2013 Oct 26.

Abstract

A 64-year-old woman developed pure red cell aplasia (PRCA) 4 years after thymectomy for thymoma. During anti-thymocyte globulin treatment, the patient developed cytomegalovirus pneumonia and was thus unable to continue immunosuppressive therapy and became transfusion dependent. Deferasirox was started for treatment with iron overload when serum ferritin increased to >1000 ng/mL. Seven months after initiation of deferasirox treatment, serum ferritin level decreased the normal range and the patient has remained transfusion independent thereafter. Deferasirox was discontinued when serum ferritin level decreased below 500 ng/mL, and she has maintained in complete remission over the last 15 months. Hypotheses have been raised regarding the improvement of hematopoiesis by deferasirox treatment, but the mechanism whereby this might be achieved remains unclear. Deferasirox treatment may be clinically beneficial both by reducing iron overload and by improving hematopoiesis in patients with PRCA.

摘要

一位 64 岁女性在因胸腺瘤行胸腺切除术 4 年后发生纯红细胞再生障碍性贫血(PRCA)。在使用抗胸腺细胞球蛋白治疗期间,该患者发生巨细胞病毒肺炎,因此无法继续进行免疫抑制治疗,并依赖输血。当血清铁蛋白升高至>1000ng/mL 时,开始使用去铁酮治疗铁过载。开始使用去铁酮治疗 7 个月后,血清铁蛋白水平降至正常范围,此后该患者无需再输血。当血清铁蛋白水平降至<500ng/mL 时,停用去铁酮,在过去的 15 个月中,她一直处于完全缓解状态。人们提出了去铁酮治疗可改善造血的假说,但尚不清楚其实现的机制。去铁酮治疗可能通过减轻铁过载和改善 PRCA 患者的造血功能而具有临床益处。

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