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噬血细胞性淋巴组织细胞增生症:一名5岁女童难治性疟疾的病因

Haemophagocytic lymphohistiocytosis: a cause of unresponsive malaria in a 5-year-old girl.

作者信息

Bhagat Manish, Kanhere Sujata, Kadakia Purvi, Phadke Varsha, George Riya, Chaudhari Kushagra

机构信息

Department of Paediatrics, K. J. Somaiya Medical College and Hospital , Mumbai, India.

出版信息

Paediatr Int Child Health. 2015;35(4):333-6. doi: 10.1080/20469047.2015.1109227. Epub 2014 Nov 19.

Abstract

A 5-year-old immunocompetent girl presented with fever, jaundice, hepatosplenomegaly and pancytopenia. The peripheral blood smear demonstrated mixed malaria infection (Plasmodium vivax and Plasmodium falciparum). Fever was persistent despite antimalarials in the absence of any coexisting bacterial or viral infection. Laboratory findings included cytopaenia, hyperbilirubinaemia, hyperferritinaemia, hypertriglyceridaemia, hyponatraemia, deranged partial thromboplastin time, decreasing ESR and megaloblastic changes on bone marrow aspiration. A final diagnosis of haemophagocytic lymphohistiocytosis (HLH) with megaloblastic anaemia associated with severe mixed malaria was made. There was a dramatic response to corticosteroid treatment with improvement in her clinical condition. This report endorses the use of corticosteroids in malaria-associated HLH whenever there is no clinical improvement with antimalarials alone.

摘要

一名5岁免疫功能正常的女孩出现发热、黄疸、肝脾肿大和全血细胞减少。外周血涂片显示混合性疟疾感染(间日疟原虫和恶性疟原虫)。尽管使用了抗疟药,但在没有任何并存细菌或病毒感染的情况下,发热仍持续存在。实验室检查结果包括血细胞减少、高胆红素血症、高铁蛋白血症、高甘油三酯血症、低钠血症、部分凝血活酶时间紊乱、血沉降低以及骨髓穿刺显示巨幼细胞改变。最终诊断为噬血细胞性淋巴组织细胞增生症(HLH)合并巨幼细胞贫血,与严重的混合性疟疾相关。使用皮质类固醇治疗后临床状况有显著改善。本报告支持在仅使用抗疟药无临床改善的情况下,对疟疾相关的HLH使用皮质类固醇。

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