先天性角化不良中的骨髓衰竭。

Bone marrow failure in dyskeratosis congenita.

作者信息

Hanada T, Abe T, Nakazawa M, Aoki Y, Uyeno K

出版信息

Scand J Haematol. 1984 May;32(5):496-500. doi: 10.1111/j.1600-0609.1984.tb02191.x.

DOI:10.1111/j.1600-0609.1984.tb02191.x

Abstract

We report a case of dyskeratosis congenita ( DCG ) with neutropenia, lymphocytopenia and thrombocytopenia. Peripheral blood T lymphocytes (T cells) were proved to have a suppressive effect on the colony forming unit granulocyte-macrophage (CFU-GM). Splenectomy caused a transient increase of neutrophil count with the disappearance of the suppressive T cell activity. However, pancytopenia recurred without re-appearance of suppressive T cell activity.

摘要

我们报告一例先天性角化不良（DCG）伴中性粒细胞减少、淋巴细胞减少和血小板减少的病例。外周血T淋巴细胞（T细胞）被证明对粒细胞-巨噬细胞集落形成单位（CFU-GM）有抑制作用。脾切除导致中性粒细胞计数短暂增加，抑制性T细胞活性消失。然而，全血细胞减少症复发，且抑制性T细胞活性未再次出现。