CAJ | 학술논문

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。
목적：분석1례수계／NK전체세포급성백혈병（M／NKPAL）적진단과정，제고대M／NKPAL적인식。방법대1례M／NKPAL채용세포도편염색、세포화학염색방법화류식세포술진행세포형태학화면역표형분석，병응용세포유전학화PCR기술진행염색체핵형분석급T세포수체、백혈병융합기인적검측，동시결합상관문헌진행분석。결과골수원시세포위90??4％，절대부분백혈병세포형태학유사급성림파세포백혈병L2형，과양화물매염색음성，우견Auer소체。면역표형위CD34、HLA-DR、CD33、CD7、CD56、CD38양성화cyCD3약표체，이cyMPO、CD3、CD4등음성。존재염색체핵형이상，미검측도극륭성T세포수체기인중배화백혈병상관적융합기인。결론M／NKPAL림상소견，진단복잡，부의거형태학난이진단，응주의여반유수계항원표체적T림파세포백혈병、급성수세포백혈병미분화형、T／수계혼합표형급성백혈병화모세포성NK세포백혈병／림파류등상감별。
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 re-lated 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. Expres-sion of CD34, HLA-DR,CD33,CD7,CD56,CD38 and cyCD3( dim) ,but no other markers including cyMPO,CD3 and CD4 were ob-served. Cytogenetics examination revealed abnormal karyotype. Clonal T-cell recepter gene rearrangement and fusion gene were not de-tected. Conclusion M/NKPAL is considered extremely rare and diagnosis is difficult depending merely on the morphology. It is im-portant 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.