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阵发性夜间血红蛋白尿。以急性粒单核细胞白血病告终并在白血病缓解后复发。

Paroxysmal nocturnal hemoglobinuria. Termination in acute myelomonocytic leukemia and reappearance after leukemic remission.

作者信息

Hirsch V J, Neubach P A, Parker D M, Reese M H, Stone M J

出版信息

Arch Intern Med. 1981 Mar;141(4):525-7. doi: 10.1001/archinte.141.4.525.

Abstract

In a 71-year-old man, acute myelomonocytic leukemia developed six years after a diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) had been established. The classic features of PNH disappeared with the onset of the leukemia. Chemotherapy resulted in complete leukemic remission, during which time intravascular hemolysis and a positive acidified serum (Ham's) test recurred; both findings again disappeared when the leukemia recurred. To our knowledge, this is the eighth reported case of PNH terminating in acute leukemia but is the first in which reappearance of PNH has been documented with leukemic remission. The alternating pattern of the two disorders suggests that the PNH clone survivors in the bone marrow when leukemia supervenes.

摘要

一名71岁男性在确诊阵发性夜间血红蛋白尿(PNH)6年后发生急性粒单核细胞白血病。白血病发作时,PNH的典型特征消失。化疗使白血病完全缓解,在此期间血管内溶血和酸化血清(汉姆氏)试验阳性再次出现;白血病复发时,这两项表现再次消失。据我们所知,这是第八例报道的以急性白血病告终的PNH病例,但却是第一例记录到白血病缓解时PNH再次出现的病例。这两种疾病的交替模式提示,白血病发生时,PNH克隆在骨髓中存活下来。

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