Autoreactive erythroid progenitor-T suppressor cells in the pure red cell aplasia associated with thymoma and panhypogammaglobulinemia.
Autoreactive erythroid progenitor-T suppressor cells in the pure red cell aplasia associated with thymoma and panhypogammaglobulinemia.
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与胸腺瘤和全低丙种球蛋白血症相关的纯红细胞再生障碍中的自身反应性红系祖细胞 T 抑制细胞。
DOI：
10.1002/ajh.2830230211
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发表时间：
1986
影响因子：
12.8
通讯作者：
Winkelstein,A
中科院分区：
文献类型：
--
作者：
Mangan,KF;Volkin,R;Winkelstein,A
对一例梭形细胞胸腺瘤患者进行了体外红系培养研究和淋巴细胞标志物检测，该患者出现红细胞再生障碍性贫血、全丙种球蛋白血症和多发性机会性感染。在出现时，骨髓单个核细胞培养的红系祖细胞(CFUe，BFUe)显著减少。用OKT3PAN T细胞单抗通过雌激素或补体介导的裂解去除骨髓单个核细胞中的T细胞，可促进红系祖细胞的体外生长。胸腺瘤来源的骨髓或胸液T细胞在体外可抑制自体的，但不能抑制同种异体的红系祖细胞(CFUe，BFUe)增殖。红系祖细胞抑制性T细胞以OKTH+、OKT3+、OKT8+和Ia+为主，与激活的抑制性T细胞一致。患者接受环磷酰胺和皮质类固醇治疗后，骨髓淋巴细胞减少四倍，随后迅速出现网织红细胞增多症。恢复后，红系祖细胞很容易被检测到。这些研究为胸腺瘤和低丙种球蛋白血症相关的红细胞再生障碍性贫血患者中T细胞介导的红细胞生成抑制提供了新的证据。
In vitro erythroid culture studies and lymphocyte markers were performed in a patient with a spindle cell thymoma who developed red cell aplasia, panhypogammaglobulinemia, and multiple opportunistic infections. At the time of presentation, erythroid progenitor cells (CFUe, BFUe) were markedly reduced when cultured from marrow mononuclear cells. Removal of T cells from bone marrow mononuclear cells by Erosetting or complement‐mediated lysis with OKT3 pan T cell monoclonal antibody increased growth of erythroid progenitor cells in vitro. Readdition of bone marrow or pleural fluid T cells derived from the thymoma suppressed autologous, but not allogenic, erythroid progenitor cell (CFUe, BFUe) proliferation in vitro. The erythroid progenitor suppressor T cells were predominantly OKTH +, OKT3 +, OKT8 + and Ia + consistent with activated suppressor T cells. Treatment of the patient with cyclophsphamide and corticosteroids reduced marrow lymphocytes fourfold, and a prompt reticulocytosis ensued. After recovery, erythroid progenitor cells were easily detectable. These studies provide new evidence for T cell‐mediated suppression of erythropoiesis in a unique subset of patients with red cell aplasia associated with thymoma and hypogammaglobulinemia.