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免疫功能正常的成年人中表现为噬血细胞性淋巴组织细胞增生症的埃立克体病

Ehrlichiosis Presenting as Hemophagocytic Lymphohistiocytosis in an Immunocompetent Adult.

作者信息

Patel Tarang Pankaj, Beck Phillip, Chairman Dennis, Regunath Hariharan

机构信息

University of Missouri-Columbia, Department of Medicine, 1 Hospital Dr, Columbia, MO 65201, United States.

University of Missouri-Columbia, Department of Medicine, Division of Infectious Diseases, 1 Hospital Dr, Columbia, MO 65201, United States.

出版信息

IDCases. 2020 May 12;20:e00813. doi: 10.1016/j.idcr.2020.e00813. eCollection 2020.

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) is a fatal, immunologic syndrome characterized by dysregulated tissue inflammation. HLH can be either primary or secondary; with the latter typically resulting from an infection. Diagnosis requires five or more of the following: fever, splenomegaly, cytopenia, hypertriglyceridemia, hemophagocytosis via biopsy, low natural killer (NK) cell activity, elevated ferritin and soluble CD25 level (sCD25). We present a case of HLH related to ehrlichiosis. In order to mount an effective immune response against microbes such as chaffeensis, the host must have preserved NK cell function. Being that HLH Is characterized as a state of depleted NK cell function, It is crucial to investigate the role NK cell function has in the setting of HLH on the infectivity of species.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种以组织炎症失调为特征的致命性免疫综合征。HLH 可分为原发性或继发性,后者通常由感染引起。诊断需要满足以下五项或更多条件:发热、脾肿大、血细胞减少、高甘油三酯血症、通过活检发现噬血细胞现象、自然杀伤(NK)细胞活性降低、铁蛋白升高和可溶性 CD25 水平(sCD25)升高。我们报告一例与埃立克体病相关的 HLH 病例。为了对诸如查菲埃立克体等微生物产生有效的免疫反应,宿主必须保持 NK 细胞功能。鉴于 HLH 的特征是 NK 细胞功能耗竭状态,研究 NK 细胞功能在 HLH 背景下对该物种感染性的作用至关重要。

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