Loughran T P
Veterans Administration Hospital, Syracuse, NY 13210.
Blood. 1993 Jul 1;82(1):1-14.
Three distinct clinical syndromes occur in patients with increased numbers of circulating LGL. Patients with T-LGL leukemia have clonal proliferations of CD3+ LGL typically associated with chronic neutropenia and autoimmune features. NK-LGL leukemia is characterized by clonal CD3- LGL proliferation with an acute clinical presentation marked by massive hepatosplenomegaly and systemic illness. However, most patients with increased numbers of CD3- LGL do not have clinical features of NK-LGL leukemia and have a chronic clinical course. X-linked gene analyses have supported a polyclonal LGL lymphocytosis in this syndrome. Further studies are needed to determine whether clonal progression can occur in these patients.
循环中大颗粒淋巴细胞（LGL）数量增多的患者会出现三种不同的临床综合征。T-LGL白血病患者存在CD3⁺LGL的克隆性增殖，通常伴有慢性中性粒细胞减少和自身免疫特征。NK-LGL白血病的特征是克隆性CD3⁻LGL增殖，临床表现为急性，以肝脾肿大和全身疾病为显著特点。然而，大多数CD3⁻LGL数量增多的患者没有NK-LGL白血病的临床特征，病程呈慢性。X连锁基因分析支持该综合征中存在多克隆LGL淋巴细胞增多症。需要进一步研究以确定这些患者是否会发生克隆进展。
