Background: Nonleukemic myeloid sarcoma (MS) occurs rarely. Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal condition. We report a rare case of nonleukemic MS associated with HLH.
Methods: Hematologic investigation, 18F-FDG PET/CT, bone marrow aspirate and biopsy, and lymph node biopsy were performed in a 25-year-old male patient.
Results: The patient was given a short-term treatment of etoposide and dexamethasone to control HLH. Then he received chemotherapy and responded well.
Conclusions: It is important to find the underlying cause of HLH in high-risk patients. HLH can occur secondary to nonleukemic MS. Early diagnosis and treatment can improve survival.