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环孢素A治疗1例B细胞慢性淋巴细胞白血病合并纯红细胞再生障碍性贫血患者

Cyclosporin-A therapy of pure red cell aplasia in a patient with B-cell chronic lymphocytic leukemia.

作者信息

Christen R, Morant R, Fehr J

机构信息

Department of Medicine, University Hospital, Zürich, Switzerland.

出版信息

Eur J Haematol. 1989 Mar;42(3):303-7. doi: 10.1111/j.1600-0609.1989.tb00117.x.

Abstract

A 65-year-old woman with B-cell chronic lymphocytic leukemia (CLL) and pure red cell aplasia (PRCA) is described. After initial chemotherapy with three different regimens over 3 months (prednisone, chlorambucil/vincristine, cyclophosphamide, bleomycin/cyclophosphamide, etoposide, prednisone), normalization of the blood lymphocyte count was observed, but lymphocyte infiltration of the bone marrow persisted and erythropoiesis remained virtually absent. Monotherapy with cyclosporin-A (CyA) was begun. After 35 days, a marked increase in the reticulocyte count was observed, and with continuing therapy, there was a rapid increase in the hemoglobin level. Follow-up after 13 months of uninterrupted treatment with CyA revealed remission of both CLL and PRCA. CyA should be investigated further as a therapeutic modality for PRCA in patients with CLL, and such trials might provide further clues to the pathogenesis of this peculiar association.

摘要

描述了一名65岁患有B细胞慢性淋巴细胞白血病(CLL)和纯红细胞再生障碍性贫血(PRCA)的女性。在3个月内先后采用三种不同方案进行初始化疗(泼尼松、苯丁酸氮芥/长春新碱、环磷酰胺、博来霉素/环磷酰胺、依托泊苷、泼尼松)后,血液淋巴细胞计数恢复正常,但骨髓淋巴细胞浸润持续存在,红细胞生成几乎仍未恢复。开始使用环孢素A(CyA)进行单药治疗。35天后,观察到网织红细胞计数显著增加,随着治疗的持续,血红蛋白水平迅速升高。在使用CyA不间断治疗13个月后的随访显示CLL和PRCA均缓解。对于CLL患者的PRCA,应进一步研究CyA作为一种治疗方式,此类试验可能为这种特殊关联的发病机制提供更多线索。

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