Abstract
BackgroundVisceral leishmaniasis (VL) could progress to secondary hemophagocytic lymphohistiocytosis (HLH), which is a rare but life-threatening condition with poor prognosis. So far, the clinical and laboratory characteristics of VL associated HLH have not been well elucidated.Method and findingsIn this study, we retrospectively analyzed the clinical and laboratory profiles between 17 patients with VL associated HLH and 27 patients with VL alone admitted at the Beijing Friendship Hospital, Capital Medical University from May 2016 to March 2021. In addition to the identification of Leishmania infection, hemophagocytosis was identified in bone marrow in the most cases of VL associated HLH (15/17). The patients with VL associated HLH had higher chances of bleeding, hepatomegaly, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, hypofibrinogenemia, elevated secretion of soluble IL-2 receptor or lower NK cell activity compared to patients with VL only. Furthermore, patients with VL associated HLH had higher inflammation status associated with higher levels of Th1 (TNF-α, IFN-γ, IL-1beta, IL-6, IL-8, IL-12p70), Th2 (IL-4) and Th17 cytokines (IL-17, IL-23) in the peripheral blood, and higher parasite load (qPCR and parasite culture). All 27 VL cases were totally recovered after being treated with Sodium Stibogluconate, five of the 17 patients with VL associated HLH died even after timely treatment with anti-parasite and immunosuppressive chemotherapy.ConclusionWithout appropriate treatment, visceral leishmaniosis could develop to secondary HLH. The parasite culturing and qPCR detection of bone marrow samples facilitates the diagnosis of VL associated HLH in addition to other findings of HLH. Prompt treatment with anti-Leishmania and immunosuppressive chemotherapy is critical to reduce the mortality of VL associated HLH.
Highlights
Visceral leishmaniasis (VL), known as Kala-azar, is a zoonotic disease caused by the infections of Leishmania donovani or L. infantum, listed as one of the most serious neglected tropical diseases by the World Health Organization (WHO) [1]
The parasite culturing and quantitative real-time PCR (qPCR) detection of bone marrow samples facilitates the diagnosis of VL associated hemophagocytic lymphohistiocytosis (HLH) in addition to other findings of HLH
Total 17 VL-associated HLH patients were identified based on the diagnostic criteria of HLH2014, 12 of them met seven out of eight criteria, one patient met six and the rest four met the minimal five criteria
Summary
Visceral leishmaniasis (VL), known as Kala-azar, is a zoonotic disease caused by the infections of Leishmania donovani or L. infantum, listed as one of the most serious neglected tropical diseases by the World Health Organization (WHO) [1]. More than 350 million people are at risk of Leishmania infection globally with approximate 50,000 to 90,000 new cases of VL occurred worldwide annually, of which 26,000 to 65,000 died each year [2]. Without adequate and timely treatment, over 95% VL patients would die within 2 years due to the serious complications including hemophagocytic lymphohistiocytosis (HLH) or secondary infections [4]. Hemophagocytic lymphohistiocytosis is a life-threatening syndrome characterized by uncontrolled and dysfunctional activation of T lymphocytes, natural killer (NK) cells, and macrophages by continuous antigen stimulation, resulting in hypercytokinemia (cytokine storm) and immune-mediated injury of multiple organ systems [5,6]. Visceral leishmaniasis (VL) could progress to secondary hemophagocytic lymphohistiocytosis (HLH), which is a rare but life-threatening condition with poor prognosis. The clinical and laboratory characteristics of VL associated HLH have not been well elucidated
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