Methotrexate-associated lymphoproliferative disorder (MTX-LPD) was initially described by Ellemans et al. in 1991, who documented a patient who developed lymphoma during immunosuppressant treatment for autoimmune disease. 1 MTX-LPD is now recognized as an important clinical entity and includes many lymphomas that develop in patients undergoing MTX treatment for rheumatoid arthritis (RA). In 2017, the World Health Organization (WHO) subclassified this diagnosis within "other iatrogenic immunodeficiencyassociated lymphoproliferative disorders" as part of a larger category of immunodeficiency-associated lymphoproliferative disorders. 2 MTX has been used as a first-line drug for RA in Japan since 1999; this drug is currently regarded as the most effective treatment for 0.6-1 million RA patients. 3,4 RA patients are likely to develop lymphoma at a 2-4-times higher rate than that observed in the general population; RA patients undergoing treatment with MTX are 1.7-times more likely to develop LPD than those not treated using this immunomodulatory drug. 5,6 Although MTX itself is not believed to promote the development of LPDs in patients with RA, disease activity associated with this diagnosis together with MTXmediated immune suppression may induce its development. 7 The incidence of MTX-LPD is currently higher in Japan than in the United States or Europe. 8,9 There are several published reports documenting the clinicopathological characteristics of MTX-LPD in Japan. MTX-LPD includes several characteristic histological