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感染相关噬血细胞性淋巴组织细胞增生症:一种不寻常的临床伪装者。

Infection-associated Hemophagocytic Lymphohistiocytosis: An Unusual Clinical Masquerader.

作者信息

Abbas Awais, Raza Mohammad, Majid Aamina, Khalid Yumna, Bin Waqar Syed Hamza

机构信息

Department of Pediatrics, Civil Hospital Karachi, Dow University of Health Sciences (DUHS), Karachi, PAK.

Pediatric Department, Civil Hospital Karachi, Dow University of Health Sciences (DUHS), Karachi, PAK.

出版信息

Cureus. 2018 Apr 12;10(4):e2472. doi: 10.7759/cureus.2472.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) secondary to an infection is a great impersonator. It is caused by hyperimmune activation, which leads to a wide array of hematological abnormalities. If the disease is untreated, it is usually fatal. We report the case of a four-year-old girl who presented to our tertiary care hospital with high-grade fever, frequent loose stools, and bleeding from the lips and gums. Investigations showed pancytopenia, hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia whereas the bone marrow biopsy revealed hemophagocytosis with trilineage suppression. Blood cultures grew Salmonella typhi. After ruling out other possibilities, the diagnosis of HLH was made as per the HLH-2004 diagnostic criteria. The patient responded well to culture-sensitive antibiotics and supportive treatment. We discuss the diagnosis and clinical course of this unique case and strive to create awareness about secondary HLH induced by common diseases, such as typhoid fever.

摘要

感染继发的噬血细胞性淋巴组织细胞增生症(HLH)极具迷惑性。它由过度免疫激活引起,可导致一系列血液学异常。若该病未得到治疗,通常会致命。我们报告一例4岁女孩的病例,她因高热、频繁腹泻以及嘴唇和牙龈出血就诊于我们的三级医疗中心。检查显示全血细胞减少、高铁蛋白血症、低纤维蛋白原血症和高甘油三酯血症,而骨髓活检显示噬血细胞增多伴三系抑制。血培养检出伤寒沙门菌。排除其他可能性后,根据HLH - 2004诊断标准确诊为HLH。患者对培养敏感的抗生素及支持治疗反应良好。我们讨论了这一独特病例的诊断和临床过程,并努力提高对由常见疾病(如伤寒热)诱发的继发性HLH的认识。

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Infections associated with haemophagocytic syndrome.与噬血细胞综合征相关的感染
Lancet Infect Dis. 2007 Dec;7(12):814-22. doi: 10.1016/S1473-3099(07)70290-6.
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Hemophagocytic syndromes and infection.噬血细胞综合征与感染
Emerg Infect Dis. 2000 Nov-Dec;6(6):601-8. doi: 10.3201/eid0606.000608.

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