S3029 Timing of Lymphoproliferative Disorder Diagnosis in Patients With Celiac Disease

Introduction: Celiac disease (CeD) is associated with an increased risk of enteropathy-associated T-cell lymphoma (EATL) and other non-Hodgkin lymphomas (NHL), which contribute to increased mortality risk. In the general population, cases of B-cell NHL far outnumber T-cell NHL. Our aim was to compare CeD patients diagnosed with a lymphoproliferative disorder (LPD) before CeD diagnosis to those diagnosed with an LPD concurrently or after CeD diagnosis. Methods: We performed a retrospective study of adult patients seen at our tertiary referral center between 1980 and 2024. Diagnosis of CeD was confirmed by villous atrophy on duodenal biopsy and/or by positive CeD serology. LPD was defined as a lymphoid malignancy, including multiple myeloma, Hodgkin lymphoma, NHL, and refractory celiac disease type II, considered to be a precursor of EATL. Diagnosis of CeD and LPD within 1 year was considered concurrent. Results: There were 87 cases of LPD identified in 82 patients, with 4 patients developing more than 1 LPD. There were 41 cases of T-cell NHL, with 90% presenting concurrently or after CeD diagnosis. The 4 cases presenting before CeD diagnosis were cutaneous: cutaneous anaplastic lymphoma kinase-negative anaplastic large cell lymphoma, cutaneous T-cell lymphoma (2 cases), and lymphomatoid papulosis type A. There were 36 cases of B-cell NHL, with 31% presenting before and 69% presenting concurrently or after CeD diagnosis. Comparing patients diagnosed with LPD before CeD diagnosis to those diagnosed concurrently or after CeD diagnosis, we found a mean age of CeD diagnosis of 58.6 and 57.6 years (P = 0.9), respectively, and a mean age of LPD diagnosis of 48.5 and 65.5 years (P < 0.001), respectively. Classical CeD presentation was seen in 38.9% and 68.9% of patients (P = 0.021), respectively. After LPD diagnosis, the median survival of patients with B-cell NHL was longer than for those with T/NK-cell NHL (18.6 vs 9.4 years, P = 0.008). Conclusion: In this series, both B-cell and T-cell NHL cases were seen in CeD patients. Patients with B-cell NHL had longer median survival than those with T/NK-cell NHL. Patients diagnosed with LPD concurrently or after CeD diagnosis were more likely to have had a classical CeD presentation. Cases of T-cell NHL presenting before CeD diagnosis consisted of a small, heterogeneous group of cutaneous lymphomas, while the vast majority of T-cell NHLs were diagnosed concurrently or after CeD diagnosis.