Paediatric Hemophagocytic Lymphohistiocytosis: A Case Series With a Diverse Spectrum From a Resource-Limited Setting.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by cytokine storms leading to multi-organ dysfunction and is a highly fatal disease. Infectious diseases are the most common cause of secondary HLH. A wide variety of infections can lead to secondaryHLH. In this case series, we report five cases of HLH which had different therapeutic approaches and varied clinical courses, with one of them diagnosed as a rare entity of coronavirus disease 2019 (COVID-19)-associated HLH of multisystem inflammatory syndrome in children (MISC) spectrum, one case each of idiopathic HLH, staphylococcal infection-associated secondary HLH, leptospirosis with secondary HLH and dengue-associated HLH. The case of idiopathic HLH required initiation of immunosuppressive therapy but had a fatal outcome while others were treated successfully with antibiotics, steroids, intravenous immunoglobulin and supportive therapy. Our case series highlights the importance of evaluating for all possible infective causes thoroughly in HLH. Most patients can be managed without chemotherapy by treating the secondary causes of HLH, including common tropical infections and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection.