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近年来法美英(FAB)协作组将发生急性白血病之前的难治性细胞减少为定为骨髓发育不良综合征(MDS)。细胞遗传学研究表明,此类患者约半数以上有非随机的染色体异常为单克隆性疾病。其中原发性获得性铁粒幼红细胞性贫血(PASA)巳由G-6-PD同功酶研究证实,异常克隆来源于多能干细胞。作者研究了一例难治性贫血患者的血液病理学和细胞遗传学,揭示了三个不同的临床分期:第一期为慢性期(22个月)其核型为(45, XY,-7);第二期为再障期,其
In recent years, the French-American-British (FAB) collaborative group identified as refractory myelodysplastic syndrome (MDS) prior to acute leukemia. Cytogenetic studies have shown that about half of these patients have non-random chromosomal abnormalities as monoclonal diseases. Among them, primary acquired erythroblasts erythroblastic anemia (PASA) has been confirmed by G-6-PD isoenzyme study. Abnormal clones are derived from pluripotent stem cells. The authors studied the hematopathology and cytogenetics of a patient with refractory anemia and revealed three distinct clinical stages: the first was chronic (22 months) with a karyotype of (45, XY, -7) ; The second phase of aplastic anemia, it