Rapidly lethal secondary hemophagocytic lymphohistiocytosis predicted by fluorodeoxyglucose positron-emission tomography/computed tomography

Abstract

Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare disease with either an indolent or aggressive course. A 29-year-old male presented with fever, polyarthralgias, splenomegaly, retroperitoneal adenopathy, and laboratory findings consistent with Epstein–Barr-mediated sHLH. Consistent with a prior survival analysis by Kim et al ., splenic maximum standardized uptake value (SUV max ) >2.52 and bone marrow SUV max >3.13 on 18 F - fuorodeoxyglucose positron emission tomography/computed tomography ( 18 FDG-PET/CT) predicted an aggressive disease with poor treatment response. Despite optimal treatment, the patient rapidly progressed to death within 3 months of symptom onset. This case underscores the potential lethal nature of sHLH, and the evolving role of 18 FDG-PET/CT in predicting disease severity and treatment response.