Multiple-Organ Involvement in Familial Hemophagocytic Lymphohistiocytosis Type 2 Shown on FDG PET/CT

Abstract FDG PET/CT in an 18-year-old man with known familial hemophagocytic lymphohistiocytosis (HLH) type 2 demonstrated abnormally in the brain, liver, and lymph nodes. Pathology showed no sign of lymphoproliferative disease. The patient underwent the chemotherapy of HLH. Six months after chemotherapy, a follow-up FDG PET/CT did not show any abnormal 18 F-FDG activity, suggesting a complete response. The present case shows FDG PET/CT might not only be useful in staging the familial HLH but also able to evaluate the therapy response.