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噬血细胞性淋巴组织细胞增生症——成年患者的延迟诊断

Hemophagocytic lymphohistiocytosis-late diagnosis in an adult patient.

作者信息

Ng Dawn, Ghosh Nina, Hicks Lisa K

机构信息

University of Toronto, Medicine, 2nd floor, 500 University Ave, Toronto, Ontario, M5G 1V7, Canada.

出版信息

BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.05.2009.1858. Epub 2009 Aug 17.

Abstract

We present a case of hemophagocytic lymphohistiocytosis (HLH) in a previously healthy 30-year-old woman. The patient presented with features consistent with HLH: persistent fever, neurological abnormalities, lymphadenopathy, anaemia, leucopoenia and markedly elevated serum lactate dehydrogenase and ferritin levels. Diagnosis was delayed for approximately 1 month beyond presentation. Once initiated on treatment, the patient rapidly improved and was discharged from the intensive care unit and subsequently sent home. Unfortunately, she succumbed to progressive HLH 5 months after her initial presentation. This case highlights key clinical features associated with HLH to help prevent late diagnosis as delayed treatment may lead to irreversible multi-organ failure and/or death.

摘要

我们报告一例噬血细胞性淋巴组织细胞增生症(HLH),患者为一名30岁既往健康的女性。该患者表现出与HLH相符的特征:持续发热、神经功能异常、淋巴结病、贫血、白细胞减少以及血清乳酸脱氢酶和铁蛋白水平显著升高。诊断比出现症状延迟了约1个月。一旦开始治疗,患者迅速好转,从重症监护病房出院,随后被送回家。不幸的是,她在首次出现症状5个月后死于进行性HLH。本病例突出了与HLH相关的关键临床特征,以帮助预防延迟诊断,因为延迟治疗可能导致不可逆的多器官衰竭和/或死亡。

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