Journal of Clinical and Experimental Hematopathology
Online ISSN : 1880-9952
Print ISSN : 1346-4280
ISSN-L : 1346-4280
Original Article
Increased MYC expression without MYC gene translocation in patients with the diffuse large B-cell-lymphoma subtype of iatrogenic immunodeficiency-associated lymphoproliferative disorders
Nobuyuki Kabasawa Eisuke ShiozawaSo MuraiMayumi HommaYuka UesugiTomoharu MatsuiAyaka NakataShotaro ShimadaYohei SasakiYuta BabaMegumi WatanukiNana AraiShun FujiwaraYukiko KawaguchiHiroyuki TsukamotoYui UtoKouji YanagisawaNorimichi HattoriHirotaka SakaiHiroshi HaradaTsuyoshi NakamakiMasafumi TakimotoToshiko Yamochi-Onizuka
ジャーナル オープンアクセス

2021 年 61 巻 3 号 p. 120-125


Post-transplant lymphoproliferative disorder (PTLD) and other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPD) are iatrogenic lymphoproliferative disorders (LPD) that develop in association with immunosuppressive treatment in the setting of organ transplantation and autoimmune disease, respectively. Each has a spectrum of pathologies ranging from lymphoid hyperplasia to lymphoma. To clarify the characteristics of the diffuse large B-cell lymphoma (DLBCL) subtype in a cohort of 25 patients with PTLD or OIIA-LPD from our institute, we selected 13 with a histological subtype of DLBCL, including 2 cases of PTLD and 11 of OIIA-LPD. The median patient age at diagnosis was 70 years, with a female predominance. Both PTLD cases developed after kidney transplant. Of the patients with OIIA-LPD, 10 had rheumatoid arthritis, 1 had mixed connective tissue disease, and 8 were treated using methotrexate. Both of the PTLD patients and 6 of the OIIA-LPD patients had extranodal manifestations. All patients except for one were classified as having the non-germinal center B-cell (non-GCB) subtype according to the Hans algorithm. Tissue samples from 8 patients were positive for CD30 and 8 were positive for Epstein–Barr virus (EBV)-encoded small RNA. Seven patients had MYC-positive tissue samples, but none had MYC translocation. Our study suggests that extranodal manifestations and the non-GCB subtype are common, that EBV is associated with the DLBCL subtype of PTLD and OIIA-LPD, and that anti-CD30 therapy is applicable. In addition, our patients with the DLBCL subtype of PTLD and OIIA-LPD exhibited MYC overexpression without MYC translocation, suggesting an alternative mechanism of MYC upregulation.

© 2021 by The Japanese Society for Lymphoreticular Tissue Research

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