目的 对表现为噬血细胞综合征(HPS，又称噬血细胞淋巴组织细胞增多症HLH)的内脏利什曼病（VL）患儿资料分析总结，提高临床诊断及治疗水平。方法 回顾性分析2020年3月—2021年3月于甘肃省妇幼保健院住院治疗的4例儿童内脏利什曼病继发噬血细胞综合征患儿的临床资料，并与文献资料进行比较。结果 4例患儿均表现为持续发热、脾脏增大、血细胞三系不同程度降低，结合相关检查，符合HPS诊断。考虑其流行病学因素，完善VL相关检查，4例患儿rK39试验均阳性，3例骨髓涂片检出杜利什曼原虫。予以锑剂治疗，结局良好。复习近5年内VL-HLH相关文献发现，相比于原发性HLH，VL-HLH患儿可能更易出现铁蛋白增高和红细胞沉降率增高；相比于VL，VL-HLH可能更易出现全血细胞降低、高三酰甘油血症、高血清铁蛋白血症以及单核细胞绝对值降低。对于VL-HLH的治疗，两性霉素B可能比葡萄糖酸锑钠更具有高效性和安全性。结论 对于有疫区旅居史的患儿，在综合分析HPS原发病因时，应当考虑VL可能，同时行rK39试验和骨髓涂片检查，尽早准确给予对因治疗，以免病情加重及避免化疗引起的不良反应。

Objective To analyze the clinical data of children with visceral leishmaniasis (VL) presenting as hemophagocytic syndrome (HPS) in order to improve the clinical diagnosis and treatment level. Methods The clinical data of 4 children with HPS secondary to VL hospitalized from March 2020 to March 2021 were retrospectively analyzed, and were compared with the literature data. Results All the 4 patients showed persistent fever, splenomegaly and cytopenia. Combined with relevant examinations, the patients were diagnosed with HPS. Considering the epidemiological factors, the examination of VL was performed, and the results showed that rK39 test was positive in 4 cases, and Leishmanias donovani was detected by bone marrow smear in 3 cases. Sodium stibogluconate treatment in the four children was successful. A review of the literature on VL-HLH in the past 5 years showed that compared with primary HLH, children with VL-HLH had higher ferritin and increased erythrocyte sedimentation rate. Compared with VL, VL-HLH may be more prone to pancytopenia, hypertriglyceridemia, hyperserum ferriminemia, and lower absolute monocyte counts. Amphotericin B may be more efficient and safer than sodium stibogluconate for the treatment of VL-HLH.Conclusions For children with a history of staying in the epidemic area, the possibility of VL should be considered in the comprehensive analysis of the primary etiology of HPS, and rK39 test and bone marrow smear examination should be performed at the same time. Early and accurate treatment of the cause can avoid aggravation of the disease and the toxic side effects caused by chemotherapy.