Clinicopathological characteristics of T-NHL arising in patients with immunodeficiencies: a single-center case series of 25 patients and a review of the literature

Whereas for B cell lymphomas it is known that they occur more frequently in immunocompromised patients, such a relation has thus far not been clearly established for T cell lymphomas. Of 251 patients that were diagnosed with a T cell non-Hodgkin lymphoma in our center between 1999 and 2014, at least 25 were identified in immunocompromised patients. Here, we retrospectively analysed the clinical and pathological characteristics of these 25 cases. In addition, we searched the literature and present an overview of 605 previously published cases. The actual number of patients with B cell chronic lymphocytic leukemia and patients on immunosuppressive drugs for inflammatory bowel disease or rheumatoid arthritis in the total cohort of 251 patients diagnosed with T cell non-Hodgkin lymphoma was much higher than the number of patients expected to have these diseases in this cohort based on their prevalence in the general population. This, together with the large number of additional cases found in the literature, suggests that the risk of developing T cell non-Hodgkin lymphoma is increased in immunocompromised patients. Compared to T cell non-Hodgkin lymphoma in the general population these lymphomas are more often located extranodally, present at a younger age and appear to have a poor outcome. The observations made in this study should raise awareness of the possible development of T cell non-Hodgkin lymphoma in immunodeficient patients and challenge the prolonged use of immunosuppressive drugs in patients who are in clinical remission of their autoimmune disease