Haemophagocytic lymphohistiocytosis (HLH) secondary to diffuse large B-cell lymphoma: uncommon complication of a common disease.

Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterised by dysregulated immune activation, inflammation and multiorgan failure. In adults, HLH often arises secondary to an underlying trigger including infections, malignancies, autoimmune conditions, immunodeficiencies, chemotherapy or bone marrow transplantation. Diffuse large B-cell lymphoma (DLBCL) contributes to about 5-10% of malignancy-associated HLH. This case report highlights the clinical course of an elderly woman with HLH associated with DLBCL, who had an initial response in HLH with mini-R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin and prednisone) therapy. However, on lymphoma progression, she presented with a severe form of HLH leading to her death. This clinical scenario needs further research with the development of interventions based on the enhanced knowledge of underlying physiology.