Background: Individuals with celiac disease (CD) are at increased risk of lymphoproliferative malignancy (LPM). We examined if a family history of LPM or any cancer influenced the risk of LPM in individuals with CD. Methods: We identified 28,996 individuals with biopsy-verified CD (equal to villous atrophy, Marsh histopathology stage 3), of whom 616 had family history of LPM. Cox regression then estimated hazard ratios (HRs) for LPM in these 616 compared with two control groups. We also examined the risk of LPM in CD individuals with a family history of any cancer (n = 8,439). Results: During follow-up, 2/616 CD individuals with a family history of LPM, and 235/28,380 CD individuals without a family history of LPM developed LPM themselves. CD individuals with a family history of LPM were not at increased risk of LPM compared to general population controls (HR = 1.18; 95 % CI = 0.27-5.10), or compared to CD individuals without a family history of LPM (adjusted HR = 0.31; 95 % CI = 0.08-1.23). We found no increased risk of LPM in CD individuals with a family history of any cancer. Conclusion: This study found no evidence that a family history of LPM or any cancer increases the risk of future LPM in individuals with CD. Despite the large number of study participants, this study is nevertheless limited by few positive events due to a low absolute risk of LPM even in individuals with CD.
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