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作者报道3例病人,其血液中大颗粒淋巴细胞增多,慢性中性粒细胞减少,同时见克隆性染色体异常(三体8和三体14)。细胞表面标记T3~+、T8~+,HNK-1~+和低自然杀伤细胞活性用干扰素处理可增高,提示这种细胞是未成熟的自然杀伤细胞。类风湿因子、抗核抗体、抗中性粒细胞抗体、抗血小板抗体和抗红细胞抗体等多种自身抗体的存在,被认为是一种B细胞免疫调节功能的缺陷。此外,由其中一个病人的异常细胞作体外研究呈现免疫球蛋白生物合成受抑。抗中性粒细胞抗体及粒细胞-巨噬细胞克隆形成的直接细胞调节抑制物缺乏,支持中性粒细胞减少是体液免疫机制所致。
The authors report 3 patients, the blood of large particles of lymphocytes increased, chronic neutropenia, while see clonal chromosomal abnormalities (trisomy 8 and trisomy 14). T cell surface markers T3 ~ +, T8 ~ +, HNK-1 ~ + and natural killer cell activity can be increased with interferon treatment, suggesting that such cells are immature natural killer cells. The presence of multiple autoantibodies such as rheumatoid factor, antinuclear antibodies, anti-neutrophil antibodies, anti-platelet antibodies and anti-erythrocytic antibodies is considered to be a defect in the immunoregulatory function of B cells. In addition, immunoglobulin biosynthesis is inhibited by in vitro studies of abnormal cells in one of the patients. Anti-neutrophil antibodies and granulocyte-macrophage colony formation inhibitors of a direct lack of cell growth, support for neutropenia is humoral immune mechanism.