The role of A20 in the pathogenesis of lymphocytic malignancy

Autoimmune phenomena were identified in many different cases of hematological diseases and solid tumors, which may be due to alterations in the expression and function of the NF-κB signaling pathway. Recently, a number of studies have shown that the deletion or mutation of A20, a negative regulator of NF-κB, is frequently found in lymphomas, suggesting that it may be a linker between the altered immune response and leukemogenesis. The aim of this review is to summarize current findings of the A20 biological functions and its molecular mechanism as a tumor suppressor and immune regulator. The identification of A20 mutations and deletions in lymphocytic malignancy and the predictive significance of these aberrations are also reviewed.