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阵发性睡眠性血红蛋白尿症克隆在急性早幼粒细胞白血病患者中。

Paroxysmal nocturnal hemoglobinuria clone in a patient with acute promyelocytic leukemia.

机构信息

Hematology Oncology, Saint Joseph's University Medical Center, Paterson, New Jersey, USA.

出版信息

J Cancer Res Ther. 2022 Jan-Mar;18(1):294-296. doi: 10.4103/jcrt.JCRT_1176_19.

Abstract

Acute promyelocytic leukemia (APL) is a unique subtype of acute myeloid leukemia (AML), which presents with a distinct coagulopathy. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia which is clonal in nature due to somatic mutation. PNH may evolve to aplastic anemia, and more rarely, to a myelodysplastic syndrome or to AML. The literature review showed that AML is derived from the PNH clone as the leukemic cells lack the expression of glycosylphosphatidylinositol-linked proteins and PNH phenotype disappeared with the onset of acute leukemia. Herein, we report an unusual presentation of the coexistence of two clonal disorders PNH and APL. Our case contributes to the literature that AML in the setting of PNH is a separate disorder.

摘要

急性早幼粒细胞白血病(APL)是一种独特的急性髓系白血病(AML)亚型,表现为明显的凝血异常。阵发性睡眠性血红蛋白尿症(PNH)是一种获得性溶血性贫血,由于体细胞突变而具有克隆性。PNH 可能进展为再生障碍性贫血,更罕见的是骨髓增生异常综合征或 AML。文献复习表明,AML 来源于 PNH 克隆,因为白血病细胞缺乏糖基磷脂酰肌醇连接蛋白的表达,并且 PNH 表型随着急性白血病的发生而消失。在此,我们报告了两种克隆性疾病 PNH 和 APL 共存的不常见表现。我们的病例为文献提供了支持,即在 PNH 背景下的 AML 是一种独立的疾病。

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