CD10阴性的前B急性淋巴细胞白血病的临床特征以及预后分析  

Clinical features and prognosis in CDIO- pre-B acute lymphoblastic leukemia

作　　者：弓晓媛 王迎 刘兵城 魏辉 周春林 林冬 刘凯奇 魏述宁 宫本法 张广吉 刘云涛 赵邢力 李艳 顾闰夏 邱少伟 秘营昌 王建祥 

机构地区：[1]中国医学科学院、北京协和医学院血液学研究所、血液病医院白血病诊疗中心,天津300020

出　　处：《中华血液学杂志》2017年第1期17-21,共5页Chinese Journal of Hematology

基　　金：国家科技支撑计划（2014BA109812）;天津市应用基础及前沿技术研究计划（15JCZD-JC36400）

摘　　要：摘要 目的分析CD10阴性的前B急性淋巴细胞白血病（CD10-pre B-ALL）患者的临床特征和预后。 方法对6例成人CD10- pre B-ALL患者的临床和实验室资料进行回顾性分析，结合文献复习明确该类型患者的临床特征及预后。 结果CD10-pre B-ALL占ALL的1.5%（6/409），占B-ALL的1.8%（6/343），占pre B-ALL的11.5%（6/52）。6例患者均为男性，中位年龄为33.5岁，起病时中位WBC为101.78×109/L，所有患者均伴有MLL-AF4融合基因表达。5例患者经1个疗程诱导化疗即获得完全缓解（CR），1例患者经3个疗程化疗后才获得CR。2例患者在CR1期行异基因造血干细胞移植（allo-HSCT），1例患者CR后短期内即复发，在CR2期行allo-HSCT。1例患者正在等待移植。2例未移植患者1例复发死亡，1例尚处于缓解状态。 结论CD10-pre B-ALL是一类具有独特临床特征的成人ALL亚型，发生率较低，常见于男性，起病时白细胞水平较高，MLL-AF4融合基因表达率高，常规化疗具有较高的缓解率，但易复发，allo-HSCT有可能改善其预后。Objective To analyze the clinical features and prognosis of acute lymphoblastic leukemia patients with immunophenotype of CD10-pre-B （CD10 pre B-ALL）. Methods 6 adult cases with CD10 pre B-ALL immunophenotypes were analyzed retrospectively, related literatures were reviewed to clarify these kind of patients＇ clinical features and prognosis. Results CD10- pre B-ALL occurred in 1.5% of ALL, 1.8% of B-ALL and 11.5% of pre B-ALL respectively. All the 6 patients were male with the median age as 33.5 years old, the median white blood cells was 101.78×109/L, MLL-AF4 fusion transcripts were evident in all cases. Complete remission （CR） was achieved in 5 patients after first induction chemotherapy, 1 patient failed to respond to induction therapy, and got CR after 3 courses of chemotherapy. 2 patients underwent allogeneic hematopoietic stem cell transplantation （allo-HSCT） in CRy, 1 patient relapsed in the short term and underwent allo-HSCT in CR2. 1 patient was still waiting for allo-HSCT. Of the 2 patients who didn＇ t receive transplantation, 1 died following a relapse, the other remained to be in CR. Conclusions CD10- pre B-ALL was a rare but distinct subtype in adult ALL characterized by male dominance, high onset white blood cells and MLL rearrangement rate. Conventional chemotherapy produced a high response rate but more likely relapse, allo-HSCT may have the potential to improve the prognosis of these patients.

关 键 词：白血病 淋巴样 脑啡肽酶 基因重排 MLL 预后 

分 类 号：R733.71[医药卫生—肿瘤]