酷似急性早幼粒细胞白血病表现的髓系/NK细胞急性白血病

为了提高对髓系/NK细胞急性白血病的认识,减少临床误诊,本文报告1例酷似急性早幼粒细胞白血病(APL)表现的髓系/NK细胞急性白血病并结合文献进行分析。对患者进行了一系列临床检查,血细胞形态学和免疫表型分析,以及细胞遗传学和分子生物学等检测。结果表明:患者无肝脾、淋巴结肿大,贫血伴血小板减少,白细胞增高,白血病细胞酷似异常早幼粒细胞尤其是变异型M3(M3v)细胞;免疫表型主要为CD34-、HLA-DR-、CD117+、CD33+、CD13-、CD15+、CD56+、cMPO+和CD16-,伴有del(7)(q22q32)染色体异常,但无t(15;17)改变和PML/RARα融合基因阴性,全反式维甲酸(ATRA)治疗反应不佳。结论:髓系/NK细胞急性白血病临床少见,易与APL尤其是M3v混淆,应采用急性髓系白血病化疗方案进行治疗。

Myeloid/Natural Killer Cell Acute Leukemia Resembling Acute Promyelocytic Leukemia

In order to improve the recognition of myeloid/natural killer cell acute leukemia and to reduce misdiagnosis,one case of myeloid/natural killer cell acute leukemia resembling acute promyelocytic leukemia（APL） was reported and the related articles published were reviewed.A series of clinical tests,the morphologic and immunophenotypic analysis of leukemia cells,cytogenetic and molecular biological examinations were performed.The results indicated that the patient had anemia,thrombocytopenia and leucocytosis,but no evidence of lymphadenopathy and hepatosplenomegaly.The morphology of leukemia cells was similar to that of abnormal promyelocytic cells,especially the variant of M3（M3v） leukemia cells.The leukemia cells expressed CD117,CD33,CD15,CD56 and cMPO,but did not express CD34,HLA-DR,CD13 and CD16.Abnormal cytogenetics with del（7）（q22q32） was found.Neither t（15;17） nor PML/RARα gene rearrangement was detected.The patient failed to show a differentiation-induction response to all-trans retinoic acid（ATRA）.In conclusion,the myeloid/natural killer cell leukemia is extremely rare.It is very important to distinguis the disorder from APL/M3v.The patient with myeloid/natural kill cell acute leukemia should be treated with chemotherapy as acute myeloid leukemia.