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In the present study, we analysed 67 patients with OIIA\u2010LPDs, including 36 with diffuse large B\u2010cell lymphoma (DLBCL)\u2010type and 19 with Hodgkin lymphoma (HL)\u2010type. After discontinuation of immunosuppressive drugs, regression without relapse was achieved in 22 of 58 patients. Spontaneous regression was associated with Epstein\u2013Barr virus positivity in DLBCL\u2010type (<jats:italic>P<\/jats:italic>\u2009=\u20090\u00b7013). The 2\u2010year overall survival and progression\u2010free survival (PFS) at a median follow\u2010up of 32\u00b74\u2009months were 92\u00b77% and 72\u00b71% respectively. Furthermore, a significant difference in the 2\u2010year PFS was seen between patients with DLBCL\u2010type and HL\u2010type OIIA\u2010LPDs (81\u00b70% vs. 40\u00b79% respectively, <jats:italic>P<\/jats:italic>\u2009=\u20090\u00b7021). In targeted sequencing of 47 genes in tumour\u2010derived DNA from 20 DLBCL\u2010type OIIA\u2010LPD samples, histone\u2010lysine <jats:italic>N<\/jats:italic>\u2010methyltransferase 2D (<jats:italic>KMT2D<\/jats:italic>; eight, 40%) and tumour necrosis factor receptor superfamily member 14 (<jats:italic>TNFRSF14<\/jats:italic>; six, 30%) were the most frequently mutated genes. TNF alpha\u2010induced protein 3 (<jats:italic>TNFAIP3<\/jats:italic>) mutations were present in four patients (20%) with DLBCL\u2010type OIIA\u2010LPD. Cases with DLBCL\u2010type OIIA\u2010LPD harbouring <jats:italic>TNFAIP3<\/jats:italic> mutations had shorter PFS and required early initiation of first chemotherapy. There were no significant factors for spontaneous regression or response rates according to the presence of mutations. Overall, OIIA\u2010LPDs, especially DLBCL\u2010types, showed favourable prognoses.<\/jats:p>","DOI":"10.1111\/bjh.17824","type":"journal-article","created":{"date-parts":[[2021,9,24]],"date-time":"2021-09-24T22:03:34Z","timestamp":1632521014000},"page":"585-594","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Retrospective analyses of other iatrogenic immunodeficiency\u2010associated lymphoproliferative disorders in patients with rheumatic diseases"],"prefix":"10.1111","volume":"195","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9256-609X","authenticated-orcid":false,"given":"Daisuke","family":"Kaji","sequence":"first","affiliation":[{"name":"Department of Hematology Graduate School of Comprehensive Human Sciences University of Tsukuba Tsukuba Japan"},{"name":"Department of Hematology Toranomon Hospital Tokyo 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