戈谢病的非免疫性溶血及文献复习。

IF 1.4 Q2 MEDICINE, GENERAL & INTERNAL Rambam Maimonides Medical Journal Pub Date : 2021-07-20 DOI:10.5041/RMMJ.10446
Eliyakim Hershkop, Idan Bergman, Alina Kurolap, Najib Dally, Hagit Baris Feldman
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引用次数: 1

摘要

戈谢病(GD)是一种常染色体隐性遗传病,其特征是巨噬细胞中糖脑苷的积累,导致“戈谢细胞”的形成。这些细胞主要浸润肝脏、脾脏和骨髓,导致肝脾肿大、细胞减少和骨痛。GD中的贫血通常被认为是由非溶血过程引起的。虽然罕见,但据报道GD中自身免疫性溶血性贫血的发生率高于一般人群。GD患者非免疫性溶血性贫血的文献很少。我们回顾了GD的溶血性贫血的文献,并报告了一例继发于GD的非免疫性溶血性贫血。我们认为,这是首例确诊GD和症状性非免疫性溶血性贫血患者对GD特异性治疗有反应的病例。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Non-immune Hemolysis in Gaucher Disease and Review of the Literature.

Gaucher disease (GD) is an autosomal recessive disease characterized by the buildup of glucocerebrosides in macrophages, resulting in the formation of "Gaucher cells." These cells predominantly infiltrate the liver, spleen, and bone marrow leading to hepatosplenomegaly, cytopenia, and bone pain. Anemia in GD is typically considered to result from non-hemolytic processes. Although rare, a higher rate of hemolytic anemia of the autoimmune type has been reported in GD than in the general population. The literature on non-immune hemolytic anemia in GD is scarce. We review the literature on hemolytic anemia in GD and report on a case of non-immune hemolytic anemia secondary to GD. We believe this is the first description of a patient with confirmed GD and symptomatic non-immune hemolytic anemia that responded to GD-specific treatment.

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来源期刊
Rambam Maimonides Medical Journal
Rambam Maimonides Medical Journal MEDICINE, GENERAL & INTERNAL-
CiteScore
3.20
自引率
6.70%
发文量
55
审稿时长
8 weeks
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