Hemophagocytic syndromes and infection.

Hemophagocytic syndromes and infection.
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DOI:
10.3201/eid0606.000608
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发表时间:
2000-11
影响因子:
11.8
通讯作者:
Fisman DN
Fisman DN
中科院分区:
医学2区
文献类型:
--
作者:
Fisman DN

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噬血细胞性淋巴组织细胞增多症(HLH)是一种罕见的综合征,其特征是发热、脾肿大、黄疸,以及骨髓和其他组织中的噬血细胞现象(巨噬细胞吞噬红细胞、白细胞、血小板及其前体)。HLH的诊断可能与恶性、遗传或自身免疫性疾病有关,但也与eb病毒感染密切相关。EBV感染的T淋巴细胞产生大量细胞因子,包括干扰素γ和肿瘤坏死因子α,可能在HLH的发病机制中发挥作用。ebv相关的HLH可能与t细胞淋巴瘤相似,可以用细胞毒性化疗治疗,而与非病毒性病原体相关的噬血细胞综合征通常对潜在感染的治疗有反应。
Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever, splenomegaly, jaundice, and the pathologic finding of hemophagocytosis (phagocytosis by macrophages of erythrocytes, leukocytes, platelets, and their precursors) in bone marrow and other tissues. HLH may be diagnosed in association with malignant, genetic, or autoimmune diseases but is also prominently linked with Epstein-Barr (EBV) virus infection. Hyperproduction of cytokines, including interferon-gamma and tumor necrosis factor-alpha, by EBV- infected T lymphocytes may play a role in the pathogenesis of HLH. EBV-associated HLH may mimic T-cell lymphoma and is treated with cytotoxic chemotherapy, while hemophagocytic syndromes associated with nonviral pathogens often respond to treatment of the underlying infection.
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发表时间: 1952-01-01
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