目的:提高对纯红系白血病(PEL)的认识。方法:报道了1例骨髓增生异常综合-难治性血细胞减少伴原始细胞增多2型(MDS-RAEB2)转化的PEL并进行相关文献复习。结果:按WHO诊断分型标准为M6b。患者贫血症状显著,全血细胞减少。骨髓红系细胞比例≥80%伴形态异常,原红细胞呈现显著巨幼样变,胞质强嗜碱性、无粒、空泡易见且常成串出现,PAS强阳性,POX阴性。白血病细胞表达CD36和血型糖蛋白A阳性,部分表达CD117、CD71阳性,缺乏髓系相关抗原表达。出现染色体异常。治疗采用蒽环类药物加阿糖胞苷的化疗方案,未缓解,放弃治疗。结论:PEL罕见,传统化疗疗效差,预后差。 Objective: To improve the understanding of pure erythroleukemia (PEL). METHODS: One case of PML with a report of myelodysplastic syndrome - refractory cytopenia and MDS-RAEB2 was reported and reviewed. Results: According to the WHO diagnostic typing standard M6b. Patients with significant anemia, pancytopenia. Bone marrow erythroid cells with a ratio of ≥ 80% with abnormal morphology, mesenchymal cells showed significant changes in metaplasia, cytoplasm basophilic, no particles, vacuoles are easy to see and often appear in strings, PAS strong positive, POX negative. Leukemic cells expressed CD36 and glycophorin A positive, partially expressed CD117, CD71 positive, the lack of myeloid-associated antigen expression. Chromosome abnormalities occur. Treatment with anthracycline plus cytarabine chemotherapy, did not ease, to give up treatment. Conclusion: PEL rare, poor efficacy of traditional chemotherapy, poor prognosis.