Lymphoma-associated hemophagocytic lymphohistiocytosis.

A 34-year-old male with a history of T cell–rich B-cell lymphoma relapsed 1 year after high-dose therapy with recurrent fevers, abdominal pain, and jaundice. He was cachetic with hepatosplenomegaly and ascites. Laboratory findings showed a white blood cell count of 2.7 109/L, hemoglobin 8.7 g/dL, platelets of 21 109/L, hyperbilirubinemia, and no evidence of hemolysis including a negative Coombs test. Bone marrow examination revealed marked hypercellularity and affirmed recurrent lymphoma (occupying 30%-40% of marrow). In addition, there was dysplasia of red cell precursors (panel A) and phagocytosis of bone marrow elements by lymphohistiocytes (panel B). A diagnosis of hemophagocytic lymphohistiocytosis (HLH) was made. Laboratory data consistent with HLH included albumin of 1.7 gm/dL, fibrinogen 65 mg/dL, ferritin 15 000 ng/mL, and soluble IL-2 receptor 6500 units/mL. The relapsed lymphoma was successfully treated with salvage therapy. A repeat bone marrow examination showed no lymphoma, normal hematopoiesis, an absence of dysplasia, and no phagocytosis. Several months later the patient relapsed and died.