T幼淋巴细胞白血病遗传学及诊疗的研究进展

Research Advances of Genetics, Diagnosis and Treatment of T-cell Prolymphocytic Leukemia——Review

T幼淋巴细胞白血病(T-PLL)是一种罕见但具有侵袭性、恶性程度极高的成熟T淋巴肿瘤,综合患者的遗传学特征、临床表现、细胞形态及免疫表型是诊断T-PLL的主要手段。目前,临床治疗目的主要是提高患者的应答率和延长缓解期。近几年,随着新型分子生物学技术的发展,研究者对于T-PLL发病和相关的遗传学机制有了更为深刻的认识,针对T-PLL的靶向药物,包括HDAC抑制剂、JAK/STAT抑制剂、AKT抑制剂、BCL-2抑制剂也在不断研发中,为改善T-PLL治疗效果提供了新的机遇。本文就T-PLL遗传学及诊疗进展作一综述。

T-cell prolymphocytic leukemia(T-PLL) is a rare but highly aggressive and malignant mature T-lymphoid tumor. The diagnosis of T-PLL mainly depend on genetic characteristics, clinical manifestations, cell morphology and immunophenotype. At present, clinical treatment is mainly aimed at improving the response rate and prolonging the remission period. With the development of new molecular biology technologies, researchers have gained a deeper understanding of the pathogenesis and related genetics of T-PLL, targeted drugs, including HDAC inhibitors, JAK/STAT inhibitors, AKT inhibitors and BCL-2 inhibitors, are also under evolution and providing the new opportunities to improve the efficacy of therapy. In this review, the advances in genetics and treatment of T-PLL were summarized briefly.