A Brief Report and Mini-Review of Visceral Leishmaniasis-associated Hemophagocytic Lymphohistiocytosis in Children

Mantadakis, Elpis; Alexiadou, Sonia; Totikidis, George; Grapsa, Anastasia; Chatzimichael, Athanassios

doi:10.1097/mph.0000000000001747

Cited by 3 publications

(5 citation statements)

References 26 publications

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“…However, it is not well characterized for the clinical and laboratory features of the visceral leishmaniasis associated HLH. Here we analyzed the clinical characterization of VL associated HLH compared with visceral leishmaniasis only, to better understand this serious complication of Leishmania infection [4,14,15,19] VL associated HLH was diagnosed based on the diagnostic criteria for both HLH and leishmaniasis. The most typical findings of HLH are hemophagocytosis, fever, hepatosplenomegaly and cytopenias.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Leishmania infection is a well-known cause of infection associated HLH [4,[8][9][10][11]. It was reported that about 25.77% of patients with adult hemophagocytic syndrome were induced by Leishmania infection [12].…”

Section: Introductionmentioning

confidence: 99%

“…However, the number of infected people are seriously under-estimated with only 20–45% reported to WHO [ 1 ]. 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 ].…”

Section: Introductionmentioning

confidence: 99%

“…It was reported that about 25.77% of patients with adult hemophagocytic syndrome were induced by Leishmania infection [ 12 ]. As many as 41.7% of children with visceral leishmaniasis developed HLH if lack of timely treatment and the mortality rate was as high as 100% [ 4 , 9 ]. The clinical features of VL overlap with that of HLH that challenges the differential diagnosis of VL and VL associated HLH especially in endemic areas of VL.…”

Section: Introductionmentioning

confidence: 99%

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Clinical and laboratory characteristics of hemophagocytic lymphohistiocytosis induced by Leishmania infantum infection

Shi

Huang

et al. 2021

PLoS Negl Trop Dis

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Background Visceral 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 findings In 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. Conclusion Without 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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Clinical and laboratory characteristics of hemophagocytic lymphohistiocytosis induced by Leishmania infantum infection

Shi

Huang

et al. 2021

PLoS Negl Trop Dis

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Parasitic and Fungal Triggers of Cytokine Storm Syndrome

Sterba,

Sterba

2024

Advances in Experimental Medicine and Biology

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Immune response to viscerotropic Leishmania: a comprehensive review

Lodi,

Voarino,

Stocco

et al. 2024

Front. Immunol.

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L. donovani and L. infantum infections are associated with a broad clinical spectrum, ranging from asymptomatic cases to visceral leishmaniasis (VL) with high mortality rates. Clinical manifestations such as post-kala-azar dermal leishmaniasis (PKDL) and visceral leishmaniasis-associated hemophagocytic lymphohistiocytosis-mimic (VL-associated HLH-mimic) further contribute to the diversity of clinical manifestations. These clinical variations are intricately influenced by the complex interplay between the host’s immune response and the parasite’s escape mechanisms. This narrative review aims to elucidate the underlying immunological mechanisms associated with each clinical manifestation, drawing from published literature within the last 5 years. Specific attention is directed toward viscerotropic Leishmania sinfection in patients with inborn errors of immunity and acquired immunodeficiencies. In VL, parasites exploit various immune evasion mechanisms, including immune checkpoints, leading to a predominantly anti-inflammatory environment that favors parasite survival. Conversely, nearly 70% of individuals are capable of mounting an effective pro-inflammatory immune response, forming granulomas that contain the parasites. Despite this, some patients may experience reactivation of the disease upon immunosuppression, challenging current understandings of parasite eradication. Individuals living with HIV and those with inborn errors of immunity present a more severe course of infection, often with higher relapse rates. Therefore, it is crucial to exclude both primary and acquired immune deficiencies in patients presenting disease relapse and VL-associated HLH-mimic. The distinction between VL and HLH can be challenging due to clinical similarities, suggesting that the nosological entity known as VL-associated HLH may represent a severe presentation of symptomatic VL and it should be considered more accurate referring to this condition as VL-associated HLH-mimic. Consequently, excluding VL in patients presenting with HLH is essential, as appropriate antimicrobial therapy can reverse immune dysregulation. A comprehensive understanding of the immune-host interaction underlying Leishmania infection is crucial for formulating effective treatment and preventive strategies to mitigate the disease burden.

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A Brief Report and Mini-Review of Visceral Leishmaniasis-associated Hemophagocytic Lymphohistiocytosis in Children

Cited by 3 publications

References 26 publications

Clinical and laboratory characteristics of hemophagocytic lymphohistiocytosis induced by Leishmania infantum infection

Clinical and laboratory characteristics of hemophagocytic lymphohistiocytosis induced by Leishmania infantum infection

Parasitic and Fungal Triggers of Cytokine Storm Syndrome

Immune response to viscerotropic Leishmania: a comprehensive review

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