{"title":"Factors Predicting Spontaneous Regression in Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Diseases.","authors":"Kosuke Takayama, Yuki Nakajima, Takuya Miyazaki, Kenji Matsumoto, Haruka Yamanokawa, Yuki Yamada, Kohei Shinmura, Yuto Hibino, Mayoko Shirafuta, Jun Nukui, Marika Tanaka, Haruka Teshigawara, Yoshimi Ishii, Maki Hagihara, Shin Fujisawa, Hideaki Nakajima","doi":"10.1016/j.clml.2025.02.008","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Other iatrogenic immunodeficiency-associated lymphoproliferative diseases (OIIA-LPDs) occur in patients taking immunosuppressive drugs (ISDs) for autoimmune diseases, such as rheumatoid arthritis and have been suggested to be associated with Epstein-Barr virus (EBV) infection. Although some patients regress spontaneously upon discontinuation of ISDs, factors predicting spontaneous regression (SR) remain controversial. Therefore, we conducted a retrospective observational study of the clinical characteristics and factors associated with treatment response, prognosis, and SR in patients diagnosed with OIIA-LPD.</p><p><strong>Patients and methods: </strong>We analyzed 82 patients at two institutions between 2002 and 2022, 41 (50%) of whom had SR after discontinuation of ISDs, with a 5-year overall survival (OS) rate of 86.3% and a median follow-up of 48 months (range, 9-201 months).</p><p><strong>Results: </strong>The 5-year survival rates of the SR and non-SR groups were 96.9% and 77.2%, respectively. This value was significantly higher in the SR group (P = .001). The 5-year progression-free survival (PFS) rate for all patients was 60.1%, whereas the PFS rate for patients in the non-SR group who required chemotherapy was 54.4%. In univariate analysis, localized stage, good performance status, positive EBV-encoded RNA in situ hybridization (EBER-ISH) results, low C-reactive protein level, and low soluble interleukin-2 receptor (sIL-2R) level were associated with SR. Multivariate analysis revealed that EBER-ISH positivity and low sIL-2R levels were associated with SR (P = .016 and .012, respectively).</p><p><strong>Conclusion: </strong>The OS was significantly longer in the SR group than in the non-SR group. EBER-ISH and sIL-2R levels are predictors of SR.</p>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":" ","pages":""},"PeriodicalIF":2.7000,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Lymphoma, Myeloma & Leukemia","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.clml.2025.02.008","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Other iatrogenic immunodeficiency-associated lymphoproliferative diseases (OIIA-LPDs) occur in patients taking immunosuppressive drugs (ISDs) for autoimmune diseases, such as rheumatoid arthritis and have been suggested to be associated with Epstein-Barr virus (EBV) infection. Although some patients regress spontaneously upon discontinuation of ISDs, factors predicting spontaneous regression (SR) remain controversial. Therefore, we conducted a retrospective observational study of the clinical characteristics and factors associated with treatment response, prognosis, and SR in patients diagnosed with OIIA-LPD.
Patients and methods: We analyzed 82 patients at two institutions between 2002 and 2022, 41 (50%) of whom had SR after discontinuation of ISDs, with a 5-year overall survival (OS) rate of 86.3% and a median follow-up of 48 months (range, 9-201 months).
Results: The 5-year survival rates of the SR and non-SR groups were 96.9% and 77.2%, respectively. This value was significantly higher in the SR group (P = .001). The 5-year progression-free survival (PFS) rate for all patients was 60.1%, whereas the PFS rate for patients in the non-SR group who required chemotherapy was 54.4%. In univariate analysis, localized stage, good performance status, positive EBV-encoded RNA in situ hybridization (EBER-ISH) results, low C-reactive protein level, and low soluble interleukin-2 receptor (sIL-2R) level were associated with SR. Multivariate analysis revealed that EBER-ISH positivity and low sIL-2R levels were associated with SR (P = .016 and .012, respectively).
Conclusion: The OS was significantly longer in the SR group than in the non-SR group. EBER-ISH and sIL-2R levels are predictors of SR.
期刊介绍:
Clinical Lymphoma, Myeloma & Leukemia is a peer-reviewed monthly journal that publishes original articles describing various aspects of clinical and translational research of lymphoma, myeloma and leukemia. Clinical Lymphoma, Myeloma & Leukemia is devoted to articles on detection, diagnosis, prevention, and treatment of lymphoma, myeloma, leukemia and related disorders including macroglobulinemia, amyloidosis, and plasma-cell dyscrasias. The main emphasis is on recent scientific developments in all areas related to lymphoma, myeloma and leukemia. Specific areas of interest include clinical research and mechanistic approaches; drug sensitivity and resistance; gene and antisense therapy; pathology, markers, and prognostic indicators; chemoprevention strategies; multimodality therapy; and integration of various approaches.