骨髓增生异常综合征转化纯红系白血病1例并文献复习

One case of pure erythroid 1eukemia evolved from myelodysplastic syndrome and review of the literature

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 better understand the characteristic of pure erythroid 1eukemia（PEL）.Method： A rare case of pure erythroid leukemia evolved from myelodysplastic syndrome-refractory anemia with excess blasts Type-2（MDS-RAEB2）was reported the related literatures were reviewed.Result：According to the WHO diagnostic criteria,PEL is classified as M6b.Patients＇anemia symptoms are notable,most with pancytopenia.Bone marrow erythroid cell ratio is more than or equal to 80% with abnormal morphology and the original red cells show significant megaloblastic amyloidosis,strong basophilic cytoplasm,no grain,visible vacuoles often occurring in clusters,PAS positive,POX negative.Leukocytes＇expression of CD36 and glycophorin A are often positive,partly CD117 positive and low expression of CD71,lacking of myeloid-associated antigen expression.Chromosomal abnormalities can be found in more than 70% patients without specific genetic alterations.-5/5q-,-7/7q-or ＋8 changes and complex karyotype abnormalities are most common.Anthracycline plus cytarabine chemotherapy regimens are more adopted.Chemotherapy complete remission（CR）rate of primary PEL is about 50%-60% and only 10%-20% in secondary PEL with short remission period and median survival time is 4-5 months.Conelusion：Pure erythroleukemia is very rare,Curative effect and prognosis are poor with the traditional chemotherapy.