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We retrospectively reviewed 38 patients with genetically or clinically confirmed IEI and persistent LPD, comparing those with nonneoplastic\/reactive hyperplasia to those who developed overt lymphoid neoplasm (lymphoma). Overall, 26% developed lymphoma\u2014predominantly classical Hodgkin lymphoma or diffuse large B cell lymphoma\u2014often after earlier IEI onset. Immunophenotyping and principal component analysis revealed that patients with common variable immunodeficiency developing Hodgkin lymphoma shared a distinctive T cell profile, differing from immunocompetent lymphoma cases. Centralized histologic re-evaluation reclassified several presumed lymphoma as nonneoplastic\/reactive hyperplasia and identified Castleman-like and germinal center transformation patterns in nonneoplastic\/reactive LPD. Notably, elevated blood IgM and circulating T follicular helper cells mirrored IgM deposits and PD-1+ T cells in lymph nodes. These findings highlight the importance of an integrated approach involving clinical, genetic, and pathological reviews to improve IEI diagnosis and avoid overtreatment.<\/jats:p>","DOI":"10.70962\/jhi.20250174","type":"journal-article","created":{"date-parts":[[2025,12,23]],"date-time":"2025-12-23T07:21:42Z","timestamp":1766474502000},"update-policy":"https:\/\/doi.org\/10.70962\/jhi.crossmarkpolicy","source":"Crossref","is-referenced-by-count":6,"title":["Lymphoproliferation in inborn errors of immunity: From challenging diagnosis to histologic revision"],"prefix":"10.70962","volume":"2","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1133-9563","authenticated-orcid":true,"given":"Mattia","family":"Moratti","sequence":"first","affiliation":[{"id":[{"id":"https:\/\/ror.org\/02p77k626","id-type":"ROR","asserted-by":"publisher"}],"name":"Molecular Medicine and Applied Biotechnology, University of Rome Tor Vergata 1 Department of Biomedicine and Prevention, , 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