与Good综合征相关的纯红细胞再生障碍伴骨髓增生异常综合征

Good's syndrome-associated pure red cell aplasia with myelodysplastic syndrome.

作者信息

Nitta Hideaki, Harada Yuka, Okikawa Yoshiko, Fujii Masayoshi, Arihiro Koji, Kimura Akiro, Harada Hironori

机构信息

Department of Hematology and Oncology, Division of Clinical Research, Research Institute for Radiation Biology and Medicine, Hiroshima University, Japan.

出版信息

Intern Med. 2011;50(18):2011-4. doi: 10.2169/internalmedicine.50.5709. Epub 2011 Sep 15.

DOI:10.2169/internalmedicine.50.5709

PMID:21921387

Abstract

We report a case of Good's syndrome-associated pure red cell aplasia (PRCA) with myelodysplastic syndrome (MDS). In this case, effector memory T (T(EM)) cells were expanded in the bone marrow. It remains uncertain whether the development of MDS was caused by the basic marrow defects or radiation therapy. However, since CD8(+) perforin(+) T(EM) cells expanded in the bone marrow, as was previously described for 3 of our patients with thymoma-associated PRCA, it is highly possible that the pathogenic mechanism of PRCA that is accompanied by thymoma is related to the expanded CD8(+) perforin(+) T(EM) cells in this MDS-complicated case.

摘要

我们报告一例与胸腺瘤相关的纯红细胞再生障碍性贫血（PRCA）合并骨髓增生异常综合征（MDS）的病例。在该病例中，效应记忆T（T(EM)）细胞在骨髓中扩增。MDS的发生是由基础骨髓缺陷还是放射治疗引起仍不确定。然而，由于骨髓中CD8(+)穿孔素(+) T(EM)细胞扩增，正如我们之前描述的3例胸腺瘤相关PRCA患者那样，在这个合并MDS的病例中，胸腺瘤伴发PRCA的致病机制很可能与扩增的CD8(+)穿孔素(+) T(EM)细胞有关。