ABSTRACT

Hemophagocytic lymphohistiocytosis (HLH) is a rapidly progressive inflammatory disorder associated with cytopenia, splenomegaly, cytokine overproduction and hyperferritinemia. It is a rare but potentially fatal disorder, particularly in developing countries like India, Bangladesh and Nepal, often associated with infections such as enteric fever. Recognising HLH is challenging due to nonspecific symptoms of fever, cytopenia, and organ dysfunction and requires prompt identification and aggressive treatment. Molecular confirmation or fulfilment of specific clinical parameters is often difficult in resource-limited settings in these South Asian countries. Here, we present two cases, one woman in her 30s and a man in his 40s, both diagnosed with secondary HLH linked to enteric fever, showcasing the diagnostic challenges. Despite constraints in molecular testing, clinical and laboratory criteria were met, supporting the importance of early antibiotic intervention and supportive care to mitigate the high mortality associated with secondary HLH.