Johnson Sarah May, Gilmour Kimberly, Samarasinghe Sujith, Bamford Alasdair
Paediatric Infectious Diseases, Great Ormond Street Hospital, London, UK.
Paediatric Infectious Diseases, Royal Free London NHS Foundation Trust, London, UK.
BMJ Case Rep. 2019 Jul 10;12(7):e228307. doi: 10.1136/bcr-2018-228307.
A 4-month-old male infant presented acutely unwell with fever. He was initially treated for sepsis but failed to improve with IV broad spectrum antibiotics. Haemophagocytic lymphohistiocytosis (HLH) was diagnosed due to his fever, pancytopenia, splenomegaly, hypertriglyceridaemia, hypofibrinogenaemia and significant hyperferritinaemia. An array of differentials for HLH including both immunological and infectious causes were considered and excluded. He had travelled to Madrid, and hence visceral leishmaniasis (VL) was suspected, but was not confirmed on the initial bone marrow aspirate (BMA) microscopy or culture. He improved with empirical treatment with dexamethasone and liposomal amphotericin B. VL was later confirmed on BMA PCR. He made a good recovery and remained well at 12 month follow-up. Non-endemic countries need rapid and sensitive VL diagnostics. A thorough travel history and high clinical index of suspicion are necessary to avoid the pitfall of treatment with intense immunosuppression recommended in treatment guidelines for HLH.
一名4个月大的男婴急性发病，身体不适且伴有发热。他最初被诊断为败血症并接受治疗，但静脉注射广谱抗生素后病情未见好转。由于发热、全血细胞减少、脾肿大、高甘油三酯血症、低纤维蛋白原血症以及显著的高铁蛋白血症，该男婴被诊断为噬血细胞性淋巴组织细胞增生症（HLH）。考虑并排除了一系列导致HLH的鉴别诊断，包括免疫性和感染性病因。他曾前往马德里，因此怀疑患有内脏利什曼病（VL），但初次骨髓穿刺抽吸（BMA）显微镜检查或培养未确诊。经验性使用地塞米松和脂质体两性霉素B治疗后，他的病情有所改善。后来通过BMA聚合酶链反应（PCR）确诊为VL。他恢复良好，在12个月的随访中保持健康。非流行国家需要快速且灵敏的VL诊断方法。详细的旅行史和高度的临床怀疑指数对于避免HLH治疗指南中推荐的强烈免疫抑制治疗陷阱十分必要。
