Malignant lymphoma: pathophysiology and current therapy.

Recent advances in the understanding of molecular pathogenesis of lymphoma have enabled us to clearly define disease entity by means of a disease-specific gene mutation and to select candidates for novel targeted therapy. In this review three different clinically relevant topics related to the molecular pathogenesis of lymphoma were covered. In the 2016 revision of the World Health Organization classification of lymphoid malignancies, firstly, disease-specific mutations such as MYD88 L265P in Waldenström macroglobulinemia and BRAF V600E in hairy cell leukemia were incorporated into diagnostic tests, and secondly, the determination of cell-of-origin in diffuse large B-cell lymphoma (DLBCL) was strongly recommended in diagnosis. Ibrutinib or lenalidomide was shown to be more effective in activated B-cell (ABC) -type-DLBCL than in germinal center B-cell type-DLBCL. Therefore, randomized trials to evaluate the efficacy of adding these agents to standard chemoimmunotherapy focused on the ABC type-DLBCL. Finally, the molecular mechanisms behind the efficacy of immune checkpoint inhibitors, such as anti-programmed cell death 1 antibody in Hodgkin lymphoma, and other lymphoma subtypes were discussed.