
Rapidly lethal secondary hemophagocytic lymphohistiocytosis predicted by fluorodeoxyglucose positron-emission tomography/computed tomography
Author(s) -
Lovemore Peter Makusha,
Darko Pucar,
Colin R. Young
Publication year - 2020
Publication title -
world journal of nuclear medicine
Language(s) - English
Resource type - Journals
eISSN - 1607-3312
pISSN - 1450-1147
DOI - 10.4103/wjnm.wjnm_73_19
Subject(s) - medicine , positron emission tomography , positron emission , nuclear medicine , hemophagocytic lymphohistiocytosis , computed tomography , bone marrow , radiology , disease
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 (SUVmax) >2.52 and bone marrow SUVmax>3.13 on 18F-fuorodeoxyglucose positron emission tomography/computed tomography (18FDG-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 18FDG-PET/CT in predicting disease severity and treatment response.