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继发于弥漫性大B细胞淋巴瘤的噬血细胞性淋巴组织细胞增生症导致急性肝衰竭的罕见病因。

A rare cause of acute liver failure due to haemophagocytic lymphohistiocytosis secondary to diffuse large B-cell lymphoma.

作者信息

Coppola Andrew, Chey Chia, O'Donovan Emma, Rahman Monira

机构信息

Department of Surgery and Cancer, Imperial College London Faculty of Medicine, London SW7 2BU, UK.

Department of Gastroenterology, Surrey and Sussex Healthcare NHS Trust, Surrey RH1 5RH, UK.

出版信息

JRSM Open. 2021 Mar 2;12(3):2054270420983623. doi: 10.1177/2054270420983623. eCollection 2021 Mar.

Abstract

Acute liver failure is a life-threatening condition commonly caused by drug-induced hepatotoxicity or viral hepatitides. However, there are a number of rarer causes such as haemophagocytic lymphohistiocytosis. Haemophagocytic lymphohistiocytosis is a syndrome of uncontrolled immune cell activation, triggered by infection or malignancy, which carries a high mortality. Whilst mild to moderate liver injury is commonly seen with haemophagocytic lymphohistiocytosis, acute liver failure has rarely been reported in adults. We present a case of a 74-year-old man with acute liver failure secondary to haemophagocytic lymphohistiocytosis triggered by undiagnosed large B-cell lymphoma. Initially treated for biliary sepsis, there was a delay in the diagnosis of haemophagocytic lymphohistiocytosis and despite initiating chemotherapy, he died soon after. This case highlights the importance of considering haemophagocytic lymphohistiocytosis as a rare cause of acute liver failure, as given the life-threatening potential of haemophagocytic lymphohistiocytosis, a prompt diagnosis may allow early initiation of chemotherapy for any chance of survival.

摘要

急性肝衰竭是一种危及生命的病症,通常由药物性肝毒性或病毒性肝炎引起。然而,还有一些较为罕见的病因,如噬血细胞性淋巴组织细胞增生症。噬血细胞性淋巴组织细胞增生症是一种由感染或恶性肿瘤引发的免疫细胞失控激活综合征,死亡率很高。虽然噬血细胞性淋巴组织细胞增生症常伴有轻至中度肝损伤,但成人急性肝衰竭鲜有报道。我们报告一例74岁男性患者,因未确诊的大B细胞淋巴瘤引发噬血细胞性淋巴组织细胞增生症,进而导致急性肝衰竭。该患者最初因胆源性脓毒症接受治疗,噬血细胞性淋巴组织细胞增生症的诊断有所延迟,尽管启动了化疗,但他很快就去世了。该病例凸显了将噬血细胞性淋巴组织细胞增生症视为急性肝衰竭罕见病因的重要性,鉴于噬血细胞性淋巴组织细胞增生症具有危及生命的可能性,及时诊断可能有助于尽早启动化疗,从而带来生存机会。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f3a4/7930656/73087a74e162/10.1177_2054270420983623-fig1.jpg

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