【摘要】 目的分析1例髓系/NK前体细胞急性白血病(M/NKPAL)的诊断过程,提高对M/NKPAL的认识。方法对1例M/NKPAL采用细胞涂片染色、细胞化学染色方法和流式细胞术进行细胞形态学和免疫表型分析,并应用细胞遗传学和PCR技术进行染色体核型分析及T细胞受体、白血病融合基因的检测,同时结合相关文献进行分析。结果骨髓原始细胞为90.4%,绝大部分白血病细胞形态学类似急性淋巴细胞白血病L2型,过氧化物酶染色阴性,偶见Auer小体。免疫表型为CD34、HLA-DR、CD33、CD7、CD56、CD38阳性和cyCD3弱表达,而cyMPO、CD3、CD4等阴性。存在染色体核型异常,未检测到克隆性T细胞受体基因重排和白血病相关的融合基因。结论 M/NKPAL临床少见,诊断复杂,仅依据形态学难以诊断,应注意与伴有髓系抗原表达的T淋巴细胞白血病、急性髓细胞白血病微分化型、T/髓系混合表型急性白血病和母细胞性NK细胞白血病/淋巴瘤等相鉴别。更多还原

【Abstract】 Objective To analyze the diagnostic process of a rare case diagnosed of myeloid/nature killer cell precursor acute leukemia( M / NKPAL) and to improve the recognition of M / NKPAL. Methods Cell morphology was analyzed by marrow smear and related cell chemical staining. Immunophenotyping of blast cells was performed by flow cytometry. Cytogenetics technique was used for karyotype analysis. PCR was applied for detection of T-cell recepter gene rearrangement or fusion gene associated with leukemia. The related articles were reviewed. Results With regard to morphology,most of leukemic cells demonstrated that 90. 4% blasts in the bone marrow were generally L2-shaped with negative reactivity of myeloperoxidase staining and Auer’s rods were occasionally found. Expression of CD34,HLA-DR,CD33,CD7,CD56,CD38 and cyCD3( dim),but no other markers including cyMPO,CD3 and CD4 were observed. Cytogenetics examination revealed abnormal karyotype. Clonal T-cell recepter gene rearrangement and fusion gene were not detected. Conclusion M / NKPAL is considered extremely rare and diagnosis is difficult depending merely on the morphology. It is important to be distinguished from T-cell acute lymphoblastic leukemia with expression of myeloid-antigen,acute myeloid leukemia with minimal differentiation,mixed-phenotype acute leukemia with T / myeloid lineage and blastic NK cell leukemia / lymphoma.更多还原