Aggressive diffuse large B-cell lymphoma with hemophagocytic lymphohistiocytosis: report of one case

Hemophagocytic lymphohistiocytosis (HLH) is a rare fatal hyperinflammatory syndrome resulting in cytokine storm and secondary multi-organ impairment. The natural killer (NK)/T-cell lymphoma is the predominant subtype in patients with lymphoma-associated hemophagocytic syndrome (LAHS) in Asia. Yet the non-Hodgkin's B-cell lymphoma is a relatively uncommon trigger of HLH. We report a case of a 64-year-old woman who had a bone marrow-spleen type of diffuse large B-cell lymphoma (DLBCL) associated with HLH. The patient presented with EBV-positive infection, significantly increased inflammatory cytokines (IL-6, IL-8, IL-10), and dramatically increased aspartate aminotransferase (AST) and total bilirubin (TB), resulting the patient's aggressive clinical course and early death. This case may not only illustrate the nonspecific manifestation and rapidly progressive characteristics of HLH but also highlight the necessity of anti-inflammatory therapy for the treatment of lymphoma-associated HLH.