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噬血细胞性淋巴组织细胞增生症作为不明原因发热伴全血细胞减少和慢性肝病的诊断考虑因素。

Hemophagocytic lymphohistiocytosis as a diagnostic consideration of fever of unknown origin with pancytopenia and chronic liver disease.

作者信息

Malik Paras, Pathania Monika, Rathaur Vyas K, Kaeley Nidhi

机构信息

Department of Medicine, All India Institute of Medical Sciences (AIIMS), Rishikesh, Uttarakhand, India.

Department of Paediatrics, Government Doon Medical College, Dehradun, Uttarakhand, India.

出版信息

J Family Med Prim Care. 2019 Apr;8(4):1504-1507. doi: 10.4103/jfmpc.jfmpc_190_19.

DOI:10.4103/jfmpc.jfmpc_190_19
PMID:31143751
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6510086/
Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a severe disorder of systemic immune dysregulation which can be primary or secondary to autoimmune disorders, malignancy, or infections. We hereby describe a case of a 23-year-old male with severe hepatitis along with pancytopenia and prolonged fever of unknown origin that developed HLH triggered by staphylococcal urinary tract infection. This is a discussion of this unusual disease and its presentation and the diagnostic difficulties which may be encountered in general clinical practice.

摘要

噬血细胞性淋巴组织细胞增生症(HLH)是一种严重的系统性免疫失调疾病,可原发或继发于自身免疫性疾病、恶性肿瘤或感染。我们在此描述一例23岁男性患者,患有严重肝炎伴全血细胞减少和不明原因的长期发热,其HLH由金黄色葡萄球菌尿路感染引发。本文讨论了这种罕见疾病及其临床表现,以及在一般临床实践中可能遇到的诊断困难。

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Women with symptoms of a urinary tract infection but a negative urine culture: PCR-based quantification of Escherichia coli suggests infection in most cases.症状符合尿路感染但尿液培养为阴性的女性:基于聚合酶链反应的大肠杆菌定量检测提示大多数情况下存在感染。
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Overwhelming primary Epstein-Barr virus infection requiring corticosteroid treatment.严重的原发性爱泼斯坦-巴尔病毒感染,需要使用皮质类固醇进行治疗。
BMJ Case Rep. 2011 Oct 28;2011:bcr0820114643. doi: 10.1136/bcr.08.2011.4643.
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HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.HLH-2004:噬血细胞性淋巴组织细胞增生症的诊断与治疗指南。
Pediatr Blood Cancer. 2007 Feb;48(2):124-31. doi: 10.1002/pbc.21039.
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Current concepts in the diagnosis and treatment of typhoid fever.伤寒热诊断与治疗的当前概念
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