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阵发性夜间血红蛋白尿症终末期发展为末端脱氧核苷酸转移酶阳性急性白血病。

Paroxysmal nocturnal hemoglobinuria terminating in TdT-positive acute leukemia.

作者信息

Katahira J, Aoyama M, Oshimi K, Mizoguchi H, Okada M

出版信息

Am J Hematol. 1983 Feb;14(1):79-87. doi: 10.1002/ajh.2830140110.

DOI:10.1002/ajh.2830140110
PMID:6573126
Abstract

A case of paroxysmal nocturnal hemoglobinuria (PNH) which developed terminal transferase (TdT)-positive leukemia 5 years after the diagnosis of PNH was studied. Most of the leukemic cells were suggestively lymphoid by cytochemistry and electron microscopy, and TdT-positive by immunofluorescence studies. The development of acute lymphoblastic leukemia during the course of PNH suggests that in PNH the clonal abnormality may involve lymphoid cells as well as myeloid cells, thus raising the possibility of the disease being a disorder of the pluripotential stem cell.

摘要

研究了1例阵发性夜间血红蛋白尿(PNH)患者,该患者在诊断PNH 5年后发生了末端转移酶(TdT)阳性白血病。通过细胞化学和电子显微镜检查,大多数白血病细胞提示为淋巴细胞,免疫荧光研究显示TdT阳性。PNH病程中发生急性淋巴细胞白血病提示,在PNH中,克隆异常可能累及淋巴细胞和髓细胞,从而增加了该疾病是多能干细胞疾病的可能性。

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