Autoimmune disorders in patients with T-cell lymphoma: a comprehensive review

Increased risk of B-cell lymphoma in patients with autoimmune diseases is well known. Medical literature also links various autoimmune conditions with T-cell lymphomas (TCLs), a rarer but very heterogeneous group of generally aggressive neoplasms.

Herein, we provide a comprehensive analysis of the available peer-reviewed literature on epidemiology, clinico-laboratory features and management of autoimmune diseases in patients with TCL. Most relevant publications were identified through searching the PubMed/Medline database for articles published from inception to June 2014.

Prevalence of various hematologic and non-hematologic autoimmune conditions in patients with TCL appears to be increased. Due to the rarity of TCL, scientific literature on autoimmune conditions in these patients consists mainly of case series and isolated reports.

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.