Hemophagocyte lymphohistiocytosis secondary to bilateral epididymal diffuse large B-cell lymphoma

We report a rare case of hemophagocytic lymphohistiocytosis (HLH) secondary to bilateral epididymal lymphoma. The patient is a man of 46 years old. He suffered fever 5 months before the admission. Physical examination shows splenohepatomegalia without lymphadenopathy. Laboratory studies revealed pancytopenia and coagulopathy, hyper ferritin level, large foamy macrophages containing other hematopoietic elements in bone marrow. After treatment with HLH-04 protocol, the patient recovered, but relapsed 1 month later and suffered pudendal pain. A color Doppler of the scrotum shows bilateral epididymis swollen. Pathological diagnosis of biopsy is diffuse large B cell lymphoma (DLBCL). After two cycles cyclophosphamide, doxorubicin, vincristine, and prednisone plus rituximab together with intrathecal injections, the tumors on the epididymides disappeared, but relapsed intracalvarium. The patient stopped treatment due to financial reasons. Hemophagocytic lymphohistiocytosis secondary lymphoma in a sanctuary site has not been reported before and should not be ignored. Transplantation is necessary for long-time survival.