摘要
T-大颗粒淋巴细胞白血病（T—LGLL）是一种较少见的T淋巴细胞增殖性疾病，其临床表现常为脾大、中性粒细胞缺乏及合并自身免疫性疾病，如类风湿关节炎（RA）。T—LGLL的发病机制是，通过慢性的抗原刺激，激活存活信号通路和抑制凋亡信号通路，维持大颗粒淋巴细胞（LGL）的长期活化状态。临床对于TLGLL的传统治疗方法主要是采用甲氨蝶呤、环磷酰胺等免疫抑制剂，但是目前一些新型药物，如托法替尼等已进入临床研究阶段。笔者拟就近年T—LGLL的临床研究进展进行综述。
T-large granular lymphocytic leukemia （T-LGLL） is a rare T lymphocyte proliferative disease, and its clinical manifestations are splenomegaty, neutropenia and autoimmune diseases such as rheumatoid arthritis （RA）. The pathogenesis of T-LGLL is to activate survival signaling pathway and inhibit apoptotic signaling pathway to maintain long-term activation of large granular lymphocytes （LGL） by chronic antigenic stimulation, Traditional treatment methods of T-LGLL are mainly immune inhibitors such as methotrexate, cyclophosphamide and other drugs, but now there are some new drugs such as tofacitinih in clinical research. Now, the clinical research progress of T-LGLL is reviewed.
出处
《国际输血及血液学杂志》
CAS
2017年第5期432-436,共5页
International Journal of Blood Transfusion and Hematology
基金
国家自然科学基金（81270637、81600145）
关键词
白血病
大颗粒淋巴细胞
发病机制
免疫抑制剂
Leukemia, large granular lymphocytic
Etiology
Immunosuppressive agents
