NK细胞肿瘤相关的侵袭性白血病的临床特征  

Clinical features of aggressive leukemia associated with natural killer cell neoplasm

作　　者：傅行财[1] 王建宁[1] 包红雨[1] 孟庆奇[1] 宋敏[1] 张柳波[1] 候艳秋[1] 

机构地区：[1]南京医科大学第二附属医院血液科,江苏南京210011

出　　处：《现代肿瘤医学》2013年第12期2806-2810,共5页Journal of Modern Oncology

摘　　要：目的:探讨与NK细胞肿瘤相关的侵袭性白血病的临床特征。方法:报告3例不同类型与NK细胞肿瘤相关的侵袭性白血病并结合文献进行讨论。结果:例1诊断为髓系/NK前体细胞急性白血病,白血病细胞形态学为母细胞性,但部分胞浆含有Auer小体,表达CD34、CD33、CD56、CD7和cyCD3,而CD3和cyMPO阴性,未检测到TCR基因重排。例2诊断为髓系/NK细胞急性白血病,其特征为白血病细胞酷似急性早幼粒细胞白血病形态,但缺乏t(15;17)染色体异常和PML/RARα融合基因阴性,免疫表型为MPO、CD33、CD56阳性,CD34、HLA-DR和CD3阴性,对全反式维甲酸诱导分化治疗无反应。例3诊断为侵袭性NK细胞白血病,肿瘤细胞表达CD2和CD56,其他NK、T/B标记为阴性,骨髓中组织细胞增生伴噬血现象,血清EBVDNA水平增高。结论:与NK细胞肿瘤相关的侵袭性白血病较为少见,易于误诊,除髓系/NK细胞急性白血病外,其他几种类型白血病预后均较差。Objective ：To explore the clinical features of aggressive leukemia associated with natural killer（NK） cell neoplasm. Methods：Three cases of different kinds of aggressive leukemia associated with NK cell neoplasm were reported and the related articles were reviewed. Results： Case 1 was diagnosed as myeloid/NK cell precursor acute leukemia. This case exhibited blastic morphologic features but cytoplasm contained Auer＇s rods. The blastic ceils ex- pressed CD34 ＋ , CD33 ＋ , CD7 ＋ , CD56 ＋ , cyCD3 ＋ , but cyMPO - and CD3-. T - cell recepter gene rearrangement was not detected. Myeloid/NK cell acute leukemia was made in case 2 by the following tumors characteristics：acute pro- myelocytic leukemia- like morphology,lack of t （15;17） and the resultant PML/RARct fusion transcript, a unique immunophenotype（ MPO ＋ , CD33 ＋ , CD56 ＋ , CD34 - , HLA - DR - and CD3 - ）, no differentiation - induction response to all - trans retinoic acid. We diagnosed case 3 as aggressive NK cell leukemia whose presentation showed positive for CD2 and CD56 of neoplastic cells without other NK,T/B cell markers and increasing of serum EBV - DNA level. The bone marrow was generally hypercellular with histiocyte proliferation and phagocytosis. Conclusion：Aggressive leuke- mia associated with NK cell neoplasm is considered extremely rare and may lead to be misdiagnosed. These disorders are associated with the poor prognosis except myeloid/NK cell acute leukemia.

关 键 词：髓系 NK前体细胞急性白血病 髓系 NK细胞急性白血病 侵袭性NK细胞白血病 鉴别诊断 

分 类 号：R733.71[医药卫生—肿瘤]