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.
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