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阵发性睡眠性血红蛋白尿这一罕见血液疾病对胃肠病学家具有深远影响：一例病例报告及文献综述

Rare Hematological Disease of Paroxysmal Nocturnal Hemoglobinuria With Profound Implications for a Gastroenterologist: A Case Report and Literature Review.

作者信息

Parvataneni Swetha, Sunkara Tagore, Gaduputi Vinaya

机构信息

Internal Medicine, Geisinger Health System, Lewistown, USA.

Internal Medicine, Mercy Medical Center, Des Moines, USA.

出版信息

Cureus. 2020 Jul 1;12(7):e8941. doi: 10.7759/cureus.8941.

DOI:10.7759/cureus.8941
PMID:32637290
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7331906/
Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare type of thrombophilia and hematopoietic stem cell disorder characterized by mutation of the X-linked PIG-A gene. Patients with PNH present either with clinical features of intravascular hemolysis or thrombosis. Visceral vein thrombosis is associated with increased mortality risk. Here, we present an extremely rare case of a young man presenting with extensive thrombosis of multiple visceral veins from PNH.

摘要

阵发性睡眠性血红蛋白尿（PNH）是一种罕见的血栓形成倾向和造血干细胞疾病，其特征为X连锁的PIG-A基因突变。PNH患者表现为血管内溶血或血栓形成的临床特征。内脏静脉血栓形成与死亡风险增加相关。在此，我们报告一例极为罕见的年轻男性病例，该患者因PNH出现多条内脏静脉广泛血栓形成。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0df8/7331906/cdfdfe22d174/cureus-0012-00000008941-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0df8/7331906/350620a4e48c/cureus-0012-00000008941-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0df8/7331906/cdfdfe22d174/cureus-0012-00000008941-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0df8/7331906/350620a4e48c/cureus-0012-00000008941-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0df8/7331906/cdfdfe22d174/cureus-0012-00000008941-i02.jpg

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本文引用的文献

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Portal Vein Thrombosis: Imaging the Spectrum of Disease With an Emphasis on MRI Features.门静脉血栓形成：疾病谱的影像学表现，重点介绍 MRI 特征。
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