纯红细胞再生障碍性贫血合并古德综合征，伴有骨髓中干细胞因子产生减少。

Pure red cell aplasia associated with Good's syndrome accompanied by decreased stem cell factor production in the bone marrow.

作者信息

Kuribayashi Kageaki, Fujimi Akihito, Kobune Masayoshi, Takimoto Rishu, Kikuchi Shohei, Iyama Satoshi, Kato Junji, Niitsu Yoshiro, Watanabe Naoki

机构信息

Department of Clinical Laboratory Medicine, Sapporo Medical University School of Medicine.

出版信息

Intern Med. 2010;49(5):377-82. doi: 10.2169/internalmedicine.49.2811. Epub 2010 Mar 1.

DOI:10.2169/internalmedicine.49.2811

PMID:20190468

Abstract

A 79-year-old Japanese woman diagnosed with pure red cell aplasia (PRCA) associated with thymoma and hypogammaglobulinemia (Good's syndrome) was successfully treated with cyclosporine-A after a thymectomy. We further studied the etiology of this case. A burst-forming unit erythroid (BFU-E) assay with SCF restored erythropoiesis in vitro. SCF production was reduced in bone marrow stromal cells; however, it was restored in vitro and in vivo after cyclosporine-A treatment.

摘要

一名79岁的日本女性被诊断为与胸腺瘤和低丙种球蛋白血症（古德综合征）相关的纯红细胞再生障碍性贫血（PRCA），胸腺切除术后用环孢素A成功治疗。我们进一步研究了该病例的病因。用干细胞因子（SCF）进行的红系爆式集落形成单位（BFU-E）测定在体外恢复了红细胞生成。骨髓基质细胞中SCF的产生减少；然而，环孢素A治疗后在体外和体内均恢复。