Blaschke J, Goeken N E, Thompson J S, Dick F R, Gingrich R D
Am J Med. 1979 May;66(5):862-6. doi: 10.1016/0002-9343(79)91138-0.
Multiple infections and severe neutropenia were found in a previously healthy 29 year old man with no history of similar syndromes in the family, drug ingestion or exposure to environmental toxins. There was no evidence at the time of presentation of diseases previously associated with agranulocytosis (e.g., neoplasia, thyrotoxicosis, chronic infection, collagen-vascular disease or leukoagglutinating antibody). His serum contained a nonagglutinating, complement-dependent, cytotoxic antibody, however, reactive with peripheral blood granulocytes from 35 per cent of normal donors. The neutropenia was not affected by steroids but resolved promptly after splenectomy. Microscopic examination of the spleen revealed ingestion of polymorphonuclear leukocytes by splenic macrophages. Family studies indicated that the target antigen was non-HLA and that the antibody was not absorbed by lymphocytes or platelets. We conclude that the agranulocytosis was autoimmune in origin and suggest that similar myeloid-specific immune responses could influence granulocyte tranfusion and bone marrow transplantation by alloimmune "rejection" that would not be avoided by matching only for HLA specificities.
在一名29岁既往健康的男性中发现了多重感染和严重中性粒细胞减少症，其家族中无类似综合征病史，无药物摄入史或接触环境毒素史。就诊时没有证据表明存在先前与粒细胞缺乏症相关的疾病（如肿瘤、甲状腺毒症、慢性感染、胶原血管疾病或白细胞凝集抗体）。然而，他的血清中含有一种非凝集性、补体依赖性细胞毒性抗体，可与35%正常供者的外周血粒细胞发生反应。中性粒细胞减少症不受类固醇影响，但脾切除术后迅速缓解。脾脏显微镜检查显示脾巨噬细胞吞噬多形核白细胞。家族研究表明，靶抗原是非HLA的，并且该抗体不会被淋巴细胞或血小板吸收。我们得出结论，粒细胞缺乏症起源于自身免疫，并表明类似的髓系特异性免疫反应可能通过同种免疫“排斥”影响粒细胞输注和骨髓移植，而仅匹配HLA特异性无法避免这种“排斥”。
