Isolated liver involvement in Langerhans cell histiocytosis: A case report

被引：0
|
作者：
Allaume, Pierre [1 ]
Meneyrol, Eric [2 ]
Bernard, Gontran [2 ]
Houssel-Debry, Pauline [3 ]
Emile, Jean-Francois [4 ,5 ]
Turlin, Bruno [1 ]
机构：
[1] Univ Rennes 1, Hop Pontchaillou, Serv Anat pathol, CHU Rennes, 2 rue Henri Le Guilloux, F-35033 Rennes 9, France
[2] Univ Rennes 1, Hop Pontchaillou, Serv radiol, CHU Rennes, 2 rue Henri Le Guilloux, F-35033 Rennes 9, France
[3] Univ Rennes 1, Hop Pontchaillou, Serv Malad foie, CHU Rennes, 2 rue Henri Le Guilloux, F-35033 Rennes 9, France
[4] Univ Versailles, Serv Anat pathol, Boulogne, France
[5] Univ Versailles, AP HP, Boulogne, France
来源：
ANNALES DE PATHOLOGIE | 2024年 / 44卷 / 01期
关键词：
Langerhans; Histiocytosis; Liver; Cholangitis; MAPK; MUTATIONS; CLASSIFICATION; DIAGNOSIS;
D O I：
10.1016/j.annpat.2023.12.001
中图分类号：
R36 [病理学];
学科分类号：
100104 ;
摘要：
Langerhans cell histiocytosis (LCH) is a disease whose physiopathology remains unclear, involving both inflammatory processes and clonal proliferation. It is observable at any given age, although about ten times more frequent in children than adults. Hepatic involvement is not rare, mostly part of a systemic disease, and linked to a poor prognosis. We report here a case of LCH with solitary hepatic involvement in a 74 year -old patient. This case demonstrated molecular anomaly of the MAPK pathway, BRAF N486 P490del. Through this observation, we precise the epidemiological and histological aspects and diagnostic criteria of this rare disease. (c) 2023 Elsevier Masson SAS. All rights reserved.
引用
收藏
页码：69 / 74
页数：6
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