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自身免疫性全血细胞减少症

Autoimmune panleukopenia.

作者信息

Cline M J, Opelz G, Saxon A, Fahey J L, Golde D W

出版信息

N Engl J Med. 1976 Dec 30;295(27):1489-93. doi: 10.1056/NEJM197612302952701.

Abstract

A patient with episodic panleukopenia involving neutrophilic granulocytes, monocytes and lymphocytes had recurrent bacterial infections and a circulating cytotoxin that injured primitive myeloid cells as well as differentiated granulocytes and mononuclear cells. The cytotoxin was an immunoglobulin with activity against mouse, as well as human, myeloid stem cells. Clinical remission during cyclophosphamide therapy was associated with disappearance of the circulating leukocytotoxin. Serum from a period of active disease was cytotoxic for the patient's own lymphocytes obtained during clinical remission. Absorption of cytotoxic serum with granulocytes removed activity against both granulocytes and lymphocytes. These studies suggest an autoimmune disease in man characterized by episodic granulocytopenia, monocytopenia and lymphocytopenia due to humoral antibody against non-HLA antigens shared by several hematopoietic cell lines.

摘要

一名患有发作性全白细胞减少症的患者,其嗜中性粒细胞、单核细胞和淋巴细胞均受累,反复发生细菌感染,且有一种循环细胞毒素,可损伤原始髓样细胞以及分化的粒细胞和单核细胞。该细胞毒素是一种免疫球蛋白,对小鼠以及人类的髓样干细胞均有活性。环磷酰胺治疗期间的临床缓解与循环白细胞毒素的消失相关。疾病活动期的血清对临床缓解期获得的患者自身淋巴细胞具有细胞毒性。用粒细胞吸收细胞毒性血清可消除对粒细胞和淋巴细胞的活性。这些研究提示,人类存在一种自身免疫性疾病,其特征为由于针对几种造血细胞系共有的非 HLA 抗原的体液抗体导致发作性粒细胞减少、单核细胞减少和淋巴细胞减少。

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