Managing visceral leishmaniasis-associated hemophagocytic lymphohistiocytosis in pediatric patients: Clinical insights and treatment strategies

Abstract

Hemophagocytic lymphohistiocytosis (HLH) secondary to visceral leishmaniasis (VL-HLH) is a severe and often fatal condition marked by systemic inflammation and immune dysfunction. In this retrospective cohort study at Shanxi Children’s Hospital, 31 pediatric patients diagnosed with VL-HLH were analyzed to assess clinical characteristics, treatment outcomes, and the potential impact of stibogluconate (SSG) + adjunctive corticosteroids. Patients exhibited multisystem involvement, including persistent fever, hepatosplenomegaly, anemia, and thrombocytopenia. Laboratory findings reflected elevated inflammation markers and immune dysregulation, with effective treatment achieved through anti-leishmanial therapy utilizing SSG. The study compared outcomes between monotherapy and SSG + corticosteroids groups, revealing no significant difference in overall recovery time but higher triglyceride levels in the SSG + corticosteroids cohort. While SSG + corticosteroids did not reduce recovery time, it potentially impacted lipid metabolism. The results underscore the need for larger-scale studies to further delineate the applicability and benefits of SSG + corticosteroids in managing VL-HLH, highlighting the significance of understanding immune activation and treatment strategies in this rare and high-risk condition.

Graphical abstract

Clinical features, immunological mechanisms, and optimized treatment strategies for pediatric visceral leishmaniasis-associated hemophagocytic lymphohistiocytosis.