原始巨核细胞性白血病十分罕见,它不包括在FAB分类之内。其特征伴有急性骨髓纤维化,全血细胞减少,血和骨髓出现原始巨核细胞,且病程相对较短。本文报道1例非典型原始巨核细胞性白血病。患者46岁,日本妇女,因呼吸困难和全身不适逐渐加重4年余而入院。体检无异常。血红蛋白9.5g,血球压积27.5%,血片示轻度红细胞大小不均和异形红细胞。白细胞2,000,分类见原始细胞6.5%,淋巴细胞40%,嗜碱0.5%,单核9.5%,中性43.5%。血小板56万,偶见巨大型。血小板ADP和肾上腺素聚集功能正常。骨髓示轻度增生低下及原始细胞17.6%,小巨核细胞和成骨细胞相对增多。骨髓活检 The original megakaryoblastic leukemia is very rare, it is not included in the FAB classification. Its characteristics are associated with acute myelofibrosis, pancytopenia, primary megakaryocytes in blood and bone marrow, and a relatively short duration of disease. This article reports a case of atypical primitive megakaryoblastic leukemia. The 46-year-old Japanese woman was admitted to hospital for more than four years because of her breathing problems and general malaise. No abnormal medical examination. Hemoglobin 9.5g, hematocrit 27.5%, blood cells showed mild red blood cell size heterogeneous and irregular red blood cells. White blood cells 2,000, classification see 6.5% of primitive cells, lymphocytes 40%, basophils 0.5%, mononuclear 9.5%, neutral 43.5%. 560,000 platelets, occasionally huge type. Platelet ADP and adrenergic aggregation function properly. Bone marrow showed mild hyperplasia and primitive cells 17.6%, small megakaryocytes and osteoblasts relative increase. Bone marrow biopsy