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一例在使用依那西普和感染爱泼斯坦-巴尔病毒情况下发生的噬血细胞性淋巴组织细胞增生症和巨噬细胞活化综合征罕见病例。

A Rare Case of Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome in Settings of Etanercept Use and Epstein-Barr Virus Infection.

作者信息

Ciprian Giulio, Khoury Jessica, Raimondo Thomas J

机构信息

Internal Medicine, Roger Williams Medical Center, Providence, USA.

Critical Care Medicine, Roger Williams Medical Center, Providence, USA.

出版信息

Cureus. 2021 Oct 5;13(10):e18507. doi: 10.7759/cureus.18507. eCollection 2021 Oct.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome in which widespread activation of the immune system leads to a state of excessive inflammation causing tissue damage. While this condition is mainly established in the pediatric population; due to its rarity, physicians often do not suspect this condition in adults. However, while diagnostic criteria are based on protocols tailored for the pediatric society, recognizing this condition in a timely manner in adults is utterly important to prevent a dismal prognosis. In instances where a concomitant rheumatological disorder is present, the syndrome is referred to as macrophage activation syndrome (MAS). This report describes a case of an adult patient who presented with mucosal bleeding and abdominal pain who was later diagnosed with MAS.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见的综合征,免疫系统的广泛激活会导致过度炎症状态,进而造成组织损伤。虽然这种病症主要发生在儿童群体中;但由于其罕见性,医生在成人中往往不会怀疑这种病症。然而,尽管诊断标准是基于为儿科制定的方案,但及时在成人中识别这种病症对于防止预后不良至关重要。在存在并发风湿性疾病的情况下,该综合征被称为巨噬细胞活化综合征(MAS)。本报告描述了一例成年患者,该患者出现黏膜出血和腹痛,后来被诊断为MAS。

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Hemophagocytic Lymphohistiocytosis.噬血细胞性淋巴组织细胞增生症
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