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噬血细胞性淋巴组织细胞增生症合并HIV阳性个体的霍奇金淋巴瘤。

Haemophagocytic lymphohistiocytosis complicating Hodgkin's lymphoma in an HIV-positive individual.

作者信息

Flew S J, Radcliffe K W

机构信息

Department of Genitourinary Medicine, Whittall Street Clinic, Birmingham B4 6DH, UK.

出版信息

Int J STD AIDS. 2010 Aug;21(8):601-3. doi: 10.1258/ijsa.2010.009547.

Abstract

We report the case of an HIV-positive man who presented with pyrexia of unknown origin. Histological specimens from an inguinal lymph node and liver biopsy gave a diagnosis of Epstein Barr virus (EBV)-positive Hodgkin's lymphoma and haemophagocytic lymphohistiocytosis (HLH), respectively. HLH is a condition characterized by proliferation of activated macrophages that phagocytose leukocytes, erythrocytes and platelets. Clinical features include splenomegaly, fever and pancytopenia, all of which have a wide differential diagnosis in HIV-positive patients. HLH can be caused by infections, malignancy, drugs or autoimmune conditions. There have been a number of reports of HLH in HIV-positive patients, and it can be seen at all stages of HIV infection. HIV, lymphomas, EBV infection and haemophagocytic syndrome have a complicated pathophysiology. Unfortunately, HLH in this setting has a particularly aggressive course, often with a poor outcome. This case highlights the need for awareness of the syndrome to ensure prompt diagnosis and instigation of appropriate treatment.

摘要

我们报告了一例HIV阳性男性患者,该患者表现为不明原因发热。腹股沟淋巴结和肝脏活检的组织学标本分别诊断为爱泼斯坦-巴尔病毒(EBV)阳性霍奇金淋巴瘤和噬血细胞性淋巴组织细胞增生症(HLH)。HLH是一种以活化巨噬细胞增殖为特征的疾病,这些巨噬细胞吞噬白细胞、红细胞和血小板。临床特征包括脾肿大、发热和全血细胞减少,在HIV阳性患者中,所有这些都有广泛的鉴别诊断。HLH可由感染、恶性肿瘤、药物或自身免疫性疾病引起。已有多项关于HIV阳性患者HLH的报道,并且在HIV感染的各个阶段都可见到。HIV、淋巴瘤、EBV感染和噬血细胞综合征具有复杂的病理生理学。不幸的是,这种情况下的HLH病程特别凶险,通常预后不良。该病例强调了提高对该综合征的认识以确保及时诊断和启动适当治疗的必要性。

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