Hemophagocytic lymphohistiocytosis in an infant with multiple triggers!!

Hemophagocytic lymphohistiocytosis (HLH) is a disorder of immune dysregulation secondary to a massive unregulated cytokine storm and its downstream consequences. HLH is being increasingly recognized as a cause of pyrexia of unknown origin, unexplained cytopenias, and hepatic dysfunction. However, this potentially treatable condition is often missed due to lack of suspicion, variable, and nonspecific presentations, inability to fulfil all the diagnostic criteria and availability of diagnostic tests in resource limited settings. Both familial and acquired forms of HLH can be triggered by multiple factors in a susceptible patient. We report a 9-month old infant who developed HLH in association with Stevens-Johnson syndrome following massive blood transfusion.