Retrospective analyses of other iatrogenic immunodeficiency‐associated lymphoproliferative disorders in patients with rheumatic diseases

• DOI: 10.1111/bjh.17824
• Publication Date: 2021-09-24T22:03:34Z
• Authors: Daisuke Kaji; Manabu Kusakabe; Mamiko Sakata‐Yan...; Kenichi Makishima; Yasuhito Suehara; Keiichiro Hattori; Yasunori Ota; Takashi Mitsuki; Mitsuhiro Yuasa; Kosei Kageyama; Yuki Taya; Aya Nishida; Kazuya Ishiwata; Shinsuke Takagi; Hisashi Yamamoto; Yuki Asano‐Mori; Yoshifumi Ubara; Koji Izutsu; Naoyuki Uchida; Atsushi Wake; Shuichi Taniguchi; Go Yamamoto; Shigeru Chiba
• Keywords: Lymphoproliferative Disorders

Other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPDs) occur in patients receiving immunosuppressive drugs for autoimmune diseases; however, their clinicopathological and genetic features remain unknown. In the present study, we analysed 67 with OIIA-LPDs, including 36 diffuse large B-cell lymphoma (DLBCL)-type 19 Hodgkin (HL)-type. After discontinuation of drugs, regression without relapse was achieved 22 58 patients. Spontaneous associated Epstein-Barr virus positivity DLBCL-type (P = 0·013). The 2-year overall survival progression-free (PFS) at a median follow-up 32·4 months were 92·7% 72·1% respectively. Furthermore, significant difference PFS seen between HL-type OIIA-LPDs (81·0% vs. 40·9% respectively, P 0·021). targeted sequencing 47 genes tumour-derived DNA from 20 OIIA-LPD samples, histone-lysine N-methyltransferase 2D (KMT2D; eight, 40%) tumour necrosis factor receptor superfamily member 14 (TNFRSF14; six, 30%) most frequently mutated genes. TNF alpha-induced protein 3 (TNFAIP3) mutations four (20%) OIIA-LPD. Cases harbouring TNFAIP3 had shorter required early initiation first chemotherapy. There no factors spontaneous or response rates according to presence mutations. Overall, especially DLBCL-types, showed favourable prognoses.