Clonal diseases of large granular lymphocytes.
Clonal diseases of large granular lymphocytes.
复制标题
DOI：
10.1182/blood.v82.1.1.bloodjournal8211
复制
发表时间：
1993-07
期刊：
影响因子：
20.3
通讯作者：
T. Loughran
中科院分区：
文献类型：
--
作者：
T. Loughran
循环 LGL 数量增加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 克隆增殖，通常与慢性中性粒细胞减少症和自身免疫特征相关。 NK-LGL 白血病的特征是克隆性 CD3-LGL 增殖，并具有以大量肝脾肿大和全身性疾病为特征的急性临床表现。然而，大多数 CD3-LGL 数量增加的患者不具有 NK-LGL 白血病的临床特征，并且具有慢性临床病程。 X 连锁基因分析支持该综合征存在多克隆 LGL 淋巴细胞增多症。需要进一步的研究来确定这些患者是否会发生克隆进展。
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.