染色体核型高度复杂混合异常的侵袭性NK细胞白血病1例及文献复习  

Aggressive Natural Killer-cell Leukemia Associated With Complicated and Compounded Chromosomal Aberrations:A Case Report and Review of Literatures

作　　者：王卓[1] 贺冠强[1] 朱莉[1] 张娜[1] 张恒[1] 徐慧珍[1] 孟力[1] 张义成[1] 刘文励[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院,武汉430030

出　　处：《内科急危重症杂志》2009年第2期98-100,112,共4页Journal of Critical Care In Internal Medicine

摘　　要：目的:提高对侵袭性NK细胞白血病(ANKL)的认识。方法:报道1例侵袭性NK细胞白血病,并对国内外相关文献进行复习,介绍ANKL的诊断与治疗进展。结果:患者持续高热、黄疸、肝脾进行性肿大、全血细胞减少,外周血及骨髓中较易找到大颗粒淋巴细胞,骨髓中NK细胞占淋巴细胞的62.76%,细胞免疫表型为:CD2(+)、表面CD3(-)、胞质CD3(+)、CD5(-)、CD7(+)、CD30(-)、CD56(+)、HLA-DR(+),TCR、IgH基因重排阴性,染色体核型为高度复杂混合异常,符合侵袭性NK细胞白血病诊断标准。对化疗反应差,于诊断后1月内死亡。结论:ANKL非常少见,仅凭临床表现及细胞形态学难以确诊,遇到疑似病例应做细胞免疫表型分析、受体基因重排、病理组织学及细胞遗传学等检查以明确诊断。ANKL对常规化疗不敏感,中位生存期小于2个月。新近报道以左旋门冬酰胺酶为主的联合化疗方案可提高疗效,异基因造血干细胞移植的研究正在进行中。Objective： To improve the recognition of aggressive natural killer-cell leukemia （ANKL）. Methods： To report a case of ANKL and with literature reviewed and to introduce the progress of diagnosis and treatment. Results： The patient presented with persistent high fever, jaundice, progressive hepatosplenomegaly and pancytope nia. Leukemic large granular lymphocytes can be easily found both in peripheral blood and bone marrow. NK cells accounted for 62. 76% of lyrnphocytes in bone marrow and immunophenotype： CD2（ ＋ ） ,surface CD3（ - ） ,cytoplasm CD3（ ＋ ） ,CDS（ - ） ,CD7（ ＋ ） ,CD30（ - ） ,CD56（ ＋ ） ,HLA-DR（ ＋ ）. T-cell receptor （TCR） and IgH gene rearrangement were not detected. The patient showed very complicated and compounded chromosomal aberrations and met the diag nostic criteria. Response to chemotherapy was poor, and the patient died within one month after diagnosis. Conclusions： ANKL is a very rare hematology malignancy with poor prognosis. It＇s difficult to confirmed with clinical manifestations and cytologic appearance. Immuneophenotype, T-cell receptor gene rearrangement, histopathological and cytogenetic exams should be done to clarify the diagnosis when ANKL is suspected. ANKL is usually refractory to conventional chemotherapy, with a median survival time of less than 2 months. Regimens based on L-Asparaginase can improve the therapeutic effect. Allogeneic hematopoietic stem cell transplantation is under research now.

关 键 词：NK细胞白血病 侵袭性 细胞免疫表型 左旋门冬酰胺酶 异基因造血干细胞移植 

分 类 号：R733.73[医药卫生—肿瘤]