Autoimmune disorders can develop prior to, during or after the onset of TCL. The diagnosis of TCL should prompt early recognition of certain autoimmune disorders if clinical suspicion exists. A dysfunctional immune response in these patients may be responsible for the occurrence of autoimmunity. Conversely, autoimmune conditions might create a favorable milieu for T-cell lymphoma pathogenesis. Therefore, their presence should increase the suspicion of TCL in an appropriate clinico-laboratory context. There is need for larger studies to assess further the TCL-autoimmunity relationship as prognosis and management of these patients can be considerably affected.