Objectives: To identify and record lymphomas of T cell origin in a single centre cohort of 110 Sjögren's syndrome (SS)-associated non-Hodgkin's lymphoma (NHL) patients, followed up from 1993 to June 2020.
Methods: We searched for patients diagnosed with T cell lymphoma among 110 SS-associated NHL cases. Demographic data, history of previous lymphoma, histologic subtype, lymphoma stage, treatment schedules, and response to therapy were documented.
Results: Among the 110 SS-associated NHL patients, we identified five NHL cases of T cell origin, all of whom were women. The median time from SS diagnosis to T cell lymphoma development was 3.25 years. They all expressed at least one adverse predictive factor for lymphoma development. Lymphoma subtypes were identified as: two peripheral T cell lymphomas not otherwise specified (NOS) lymphomas, one primary cutaneous T cell lymphoma, one T large granular lymphocyte (T-LGL) leukaemia and one angioimmunoblastic T cell lymphoma. All lymphomas were stage IV, apart from the latter case that was stage III, according to the Ann Arbor staging system. All lymphomas tested positive for T cell receptor (TCR) gamma clonal rearrangements in biopsy specimens, and two were also positive for Epstein-Barr virus-encoded RNA (EBER). Two out of five patients had previously been diagnosed with B cell lymphoma, treated with combined immunochemotherapy, and one had been previously diagnosed with lymph node benign polyclonal follicular hyperplasia.
Conclusions: SS-associated T cell lymphomas constitute a minority. Treatment with anti-CD20 monoclonal antibody (mAb) and viral infections may be implicated in their pathogenesis.