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Paroxysmal Nocturnal Hemoglobinuria in Association with Chronic Myelofibrosis

机译:与慢性骨髓纤维化联合的阵发性夜间血红蛋白

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Summary: Two cases of chronic idiopathic myelofibrosis (CEVfF) associated with paroxixmal nocturnal hemoglobinuria (PNH) are descrived. Case 1 was 60 yrs old male. He was diagnosed CIMF in 1990. Association of PNH was diagnosed in 1995. PNH clone expand gradually, frequent hemolytic episodes were observed. He died by liver failure in 1999. Case 2 was 51 yrs old male. He was daiagnosed as CIMF in 1993 and association of PNH was diagnosed in 2000. Case 2 has no typical hemolytic episode so far. Since both diseases belong to clonal diorder, it is suggested clonal evolution was occurred in CIMF and developed PNH.
机译:发明内容:介绍了与Paroxmal Nocturnal血红蛋白(PNH)相关的两种慢性发作性肌肌纤维化(CEVFF)。案例1是60岁的男性。他于1990年被诊断为CIMF.PNH协会在1995年诊断出来。PNH克隆逐渐扩张,观察到频繁的溶血发作。他在1999年被肝脏失败去世。案例2是51岁的男性。他在1993年被称为CIMF,并于2000年诊断出来的PNH协会。案例2到目前为止,案例2没有典型的溶解集。由于这两种疾病属于克隆Diorder,因此建议在CIMF中发生克隆演化并开发PNH。

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