A Case of Erdheim-Chester Disease Causing Secondary Sclerosing Cholangitis

被引：1

作者：

Kamalumpundi, Vijayvardhan ^{[1
]}

Rieth, John ^{[2
]}

Tran, Huy ^{[3
]}

Ashat, Munish ^{[3
]}

Zhang, Xiaocen ^{[3
]}

机构：

[1] Univ Iowa, Roy J & Lucille A Carver Coll Med, Iowa City, IA USA

[2] Univ Iowa Hosp & Clin, Div Hematol Oncol Blood & Marrow Transplantat, Iowa City, IA USA

[3] Univ Iowa Hosp & Clin, Dept Internal Med, Div Gastroenterol & Hepatol, Iowa City, IA 52242 USA

来源：

ACG CASE REPORTS JOURNAL | 2023年 / 10卷 / 01期

关键词：

myeloid neoplasms; histiocytosis; IgG4; disease; obstructive cholangiopathy;

D O I：

10.14309/crj.0000000000000963

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

Erdheim-Chester disease (ECD) is an exceedingly rare and aggressive disease characterized by foamy CD68 + CDa-histiocytic infiltration into multiple tissues and organs. Only 1,500 cases have been diagnosed since 1930 when ECD was first described. Biliary tract involvement of ECD has only been reported in the literature once. We report a case of ECD causing extrahepatic biliary obstruction without significant bile duct dilation, mimicking primary sclerosing cholangitis or IgG4 disease.

引用

页数：2

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