Abstract：
Visceral leishmaniasis-associated hemophagocytic lymphohistiocytosis (VL-HLH) is indistinguishable from those of HLH of other etiologies due to the overlap symptoms, posing a serious threat to life. In this study, we aimed to provide insights for early diagnosis and improve outcomes in pediatric patients with VL-HLH. We retrospectively analyzed the clinical and laboratory data of 10 pediatric patients with VL-HLH and 58 pediatric patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH). The median time from symptom onset to cytopenia in patients with VL-HLH and EBV-HLH was 11 days (interquartile range, 7-15 days) and five days (interquartile range, 3.75-9.25 days) (P = 0.005). Both groups showed liver injury and increased lactate dehydrogenase levels; however the levels of aspartate aminotransferase, alanine aminotransferase, direct bilirubin, and lactate dehydrogenase in patients with VL-HLH were significantly lower than those in patients with EBV-HLH (P < 0.05). The fibrinogen and triglyceride levels were almost normal in VL-HLH patients but were significantly altered in EBV-HLH cases ( P < 0.05). The positive rate of first bone marrow microscopy examination, anti-rK39 IgG detection, and blood metagenomic next-generation sequencing was 50%, 100%, and 100%, respectively. After VL diagnosis, eight patients were treated with sodium stibogluconate and two were treated with liposomal amphotericin B. All the patients with VL-HLH recovered. Our study demonstrates that regular triglyceride and fibrinogen levels in pediatric patients with VL-HLH may help in differential diagnosis from EBV-HLH. VL-HLH is milder than EBV-HLH, with less severe liver injury and inflammatory responses, and timely treatment with antileishmanial agents is essential to improve the outcomes of pediatric patients with VL-HLH.
摘要：
内脏利什曼病相关的噬血细胞性淋巴组织细胞增生症（VL-HLH）由于重叠症状而与其他病因的HLH难以区分，对生命构成严重威胁.在这项研究中,我们旨在为VL-HLH儿科患者的早期诊断和改善预后提供见解.我们回顾性分析了10例VL-HLH患儿和58例EB病毒相关噬血细胞性淋巴组织细胞增生症(EBV-HLH)患儿的临床和实验室资料。VL-HLH和EBV-HLH患者从症状发作到血细胞减少的中位时间为11天（四分位距，7-15天)和5天(四分位数间距，3.75-9.25天）（P=0.005）。两组均显示肝损伤和乳酸脱氢酶水平升高;然而天冬氨酸转氨酶水平,丙氨酸氨基转移酶，直接胆红素,而VL-HLH患者的乳酸脱氢酶明显低于EBV-HLH患者(P<0.05)。VL-HLH患者的纤维蛋白原和甘油三酯水平基本正常，但EBV-HLH患者的纤维蛋白原和甘油三酯水平明显改变（P<0.05）。首次骨髓镜检阳性率,抗rK39IgG检测，而血液宏基因组下一代测序是50%,100%,100%,分别。VL诊断后，8例患者接受葡萄糖酸钠治疗，2例接受脂质体两性霉素B治疗。所有VL-HLH患者均恢复。我们的研究表明，VL-HLH儿科患者的常规甘油三酸酯和纤维蛋白原水平可能有助于与EBV-HLH的鉴别诊断。VL-HLH比EBV-HLH温和，肝脏损伤和炎症反应不那么严重，及时使用抗利什曼尼药治疗对于改善VL-HLH患儿的预后至关重要。
