幼淋巴细胞白血病的治疗进展——2015年第57届美国血液学年会(ASH)  

Management of prolymphocytic leukemia——A tipsheet of 57th america hematology conference(ASH) in 2015

作　　者：田园[1] 刘辉[1] TIAN Yuan LIU Hui(Department of Hematology, BeijingHospital, Beijing 100730, China)

机构地区：[1]北京医院血液科,北京100730

出　　处：《临床药物治疗杂志》2016年第5期12-18,共7页Clinical Medication Journal

摘　　要：B细胞和T细胞幼淋巴细胞白血病是一类罕见,且预后不良的淋巴组织肿瘤,二者有着相似的临床表现,均以症状性脾脏肿大和淋巴细胞增多为特征。通过严谨评估其形态学特点,免疫表型和分子遗传学特征,可将二者加以区分,并可与其他T或B细胞白血病相鉴别。尽管部分患者可能会有不同时长的惰性期,但典型的临床表现仍是侵袭性过程。T细胞幼淋巴细胞白血病(T-PLL)的一线治疗为静脉注射阿仑单抗,而B细胞幼淋巴细胞白血病(B-PLL)的一线治疗为以嘌呤类似物为基础的化学免疫治疗。新型B细胞受体抑制剂,如依鲁替尼和艾代拉里斯,可能将在B-PLL的治疗中占有一席之地,尤其对于存在P53缺失的患者可能有效。异基因干细胞移植对符合移植条件的患者仍可考虑,并且可能是此疾病目前唯一的治愈方法。在过去几年中,许多疾病发病和进展背后的分子机制逐渐被揭示,为新型靶向治疗方法的发展提供了机遇。B-cell（B-PLL） and T-cell（T-PLL） prolymphocytic leukemias are rare, poor-prognosis lymphoid neoplasms with similar presentation, characterized by symptomatic splenomegaly and lymphocytosis. They can be distinguished from each other and from other T- and B-cell leukemias by careful evaluation of morphology, immunophenotyping, and molecular genetics. The clinical behavior is typically aggressive, although a subset of patients may have an indolent phase of variable lengths. First-line therapy for T-PLL is intravenously administration of alemtuzumab and for B-PLL is in combination purine analog-based chemo-immunotherapy. New B-cell receptor inhibitors, such as ibrutinib and idelalisib, may play a role in the management of B-PLL, especially for the patients harboring abnormalities of TP53. Allogenic stem cell transplantation should still be considered for eligible patients and may be the only current therapy capable of delivering a cure. In the past few years, many molecular mechanisms underlying disease pathogenesis and progression have been revealed and are likely to lead to the development of novel targeted approaches.

关 键 词：幼淋巴细胞白血病 阿仑单抗 依鲁替尼 艾代拉里斯 

分 类 号：R969.3[医药卫生—药理学]