髓系/自然杀伤细胞祖细胞急性白血病(附1例报道并文献复习)

目的：提高对髓系／自然杀伤细胞祖细胞(myeloid／Datural killer cell precursor，M／NKP)急性白血病的认识。方法：报告1例M／NKP急性白血病并复习相关文献。结果：M／NKP急性白血病起源于髓系和NK细胞共同的祖细胞。白血病细胞形态学特征类似急性淋巴细胞白血病L型．胞体大小不等，核圆或不规则，核仁明显，胞质苍白，无嗜天青颗粒，髓过氧化物酶(MPO)染色阴性，免疫表型特征为表达CD7，CD33，CD34，CD56和HLA-DR，其他NK细胞及T、B细胞分化抗原阴性。临床特征为髓外浸润较常见，急性髓系白血病化疗方案疗效相对较好，但预后差。结论：M／NKP急性白血病具有独特的临床和实验室特征，是有别于其他NK细胞及髓系细胞恶性疾病的一类独立疾病。

MYELOID/NATURAL KILLER CELL PRECURSOR ACUTE LEUKEMIA(ONE CASE REPORT AND LITERATURE REVIEW)

Objective: To improve the recognization of myeloid/natural killer(NK) cell precursor acute leukemia. Methods: A case of myeloid/NK cell precursor acute leukemia was reported and the related literature was reviewed. Results: Myeloid/NK cell precursor acute leukemia which might origin from common progenitor between the NK cell and myeloid lineage. Morphologly, the leukemic cells generally showed acute lymphoblatic leukemia L2- shaped,with variation in cell size, round to irregular nuclei and prominent nucleioli, pale cytoplasm, and a lack of azurophilic granules. Cytochemical myeloperoxidase staining showed negative reactivity.The leukemic cells phenotype were characterized by co- expression of CD7,CD33,CD34,CD56 and frequently HLA- DR,but without expression of other NK, T cell, and B- cell markers. Clinically,the patients were characterized by remarkable extramedullary involvement, myeloid chemosensitivity and poor prognosis. Conclusion: Myeloid/NK cell precursor acute leukemia is a distinct biologic and clinical entity, which is distinguishable from other NK cell and myeloid malignancies.