LEUKEMIA OF LARGE GRANULAR LYMPHOCYTES - ASSOCIATION WITH CLONAL CHROMOSOMAL-ABNORMALITIES AND AUTOIMMUNE NEUTROPENIA, THROMBOCYTOPENIA, AND HEMOLYTIC-ANEMIA
LEUKEMIA OF LARGE GRANULAR LYMPHOCYTES - ASSOCIATION WITH CLONAL CHROMOSOMAL-ABNORMALITIES AND AUTOIMMUNE NEUTROPENIA, THROMBOCYTOPENIA, AND HEMOLYTIC-ANEMIA
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DOI：
10.7326/0003-4819-102-2-169
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发表时间：
1985-01-01
影响因子：
39.2
通讯作者：
SLICHTER, SJ
中科院分区：
文献类型：
--
作者：
LOUGHRAN, TP;KADIN, ME;SLICHTER, SJ
3例患者出现大颗粒淋巴细胞白细胞增多和慢性中性粒细胞减少。克隆性染色体异常（8三体和14三体）和脾红髓，肝窦和骨髓的淋巴细胞浸润表明大颗粒淋巴细胞的肿瘤性质。通过干扰素治疗增强的T3+、T8+、HNK-1+表型和低自然杀伤细胞活性的证明表明白血病细胞是未成熟的自然杀伤细胞。存在多种自身抗体，包括类风湿因子和抗核抗体、抗神经元抗体、抗血小板抗体和抗红细胞抗体，提示B细胞免疫调节缺陷。体外研究显示，1例患者的异常细胞对IG生物合成的抑制受损。抗中性粒细胞抗体和缺乏直接细胞介导的抑制粒细胞-巨噬细胞集落形成支持中性粒细胞减少症的体液免疫机制。在这些患者中，脾肿大、多种自身抗体伴中性粒细胞减少和大颗粒淋巴细胞淋巴细胞增多综合征是由于未成熟自然杀伤细胞的肿瘤性增殖。
Three patients had leukocytosis of large granular lymphocytes and chronic neutropenia. Clonal chromosomal abnormalities (trisomy 8 and trisomy 14) and lymphocytic infiltration of splenic red pulp, hepatic sinusoids, and bone marrow indicated the neoplastic nature of the large granular lymphocytes. Demonstration of a T3+, T8+, HNK-1+ phenotype and low natural killer cell activity that was agumented by interferon treatment showed the leukemic cells to be immature natural killer cells. Multiple autoantibodies were present, and they included rheumatoid factor and antinuclear, antineutrophil, antiplatelet, and antierythrocyte antibodies, suggesting a defect of B-cell immunoregulation. In vitro studies showed impaired suppression of Ig biosynthesis by abnormal cells from 1 patient. Antineutrophil antibodies and absence of direct cell-mediated inhibition of granulocyte-macrophage colony formation supported a humoral immune mechanism for the neutropenia. In these patients, the syndrome of splenomegaly, multiple autoantibodies with neutropenia, and lymphocytosis of large granular lymphocytes is due to a neoplastic proliferation of immature natural killer cells.