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阵发性夜间血红蛋白尿症与急性非淋巴细胞白血病。三例呈现不同细胞类型的报告。

Paroxysmal nocturnal hemoglobinuria and acute non-lymphocytic leukemia. A report of three cases exhibiting different cytologic types.

作者信息

Krause J R

出版信息

Cancer. 1983 Jun 1;51(11):2078-82. doi: 10.1002/1097-0142(19830601)51:11<2078::aid-cncr2820511121>3.0.co;2-0.

Abstract

Three individuals with paroxysmal nocturnal hemoglobinuria (PNH) developed acute non-lymphocytic leukemia (ANLL) as a terminal event. The cytologic types were different in each case suggesting a transformation that may involve a pleuripotent stem cell. Eight previous cases of PNH terminating in acute leukemia have been reported which have also been ANLL in type. Whether PNH should be considered a myelodysplastic or myeloproliferative disorder remains to be seen. PNH has been considered a clonal disorder with several populations of erythrocytes being present. Cytogenetics in the current cases failed to reveal any karyotypic abnormalities during the time PNH was present. However, an abnormal clone appeared in two cases during the time leukemia supervened. Additional studies of PNH as a postulated clonal disorder may provide interesting knowledge for this uncommon disorder.

摘要

三名阵发性夜间血红蛋白尿(PNH)患者最终发展为急性非淋巴细胞白血病(ANLL)。每例的细胞类型各不相同,提示这种转变可能涉及多能干细胞。此前已报道过8例以急性白血病告终的PNH病例,其类型也均为ANLL。PNH是否应被视为骨髓增生异常综合征或骨髓增殖性疾病仍有待观察。PNH被认为是一种存在多种红细胞群体的克隆性疾病。在目前这些病例中,当处于PNH阶段时,细胞遗传学检查未发现任何核型异常。然而,在白血病发生时,有两例出现了异常克隆。将PNH作为一种假定的克隆性疾病进行更多研究,可能会为这种罕见疾病提供有趣的认识。

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