Ricardo Kosch , Christoph Schaefers , Christian Frenzel, Walter Fiedler, Katja Weisel, Carsten Bokemeyer, Christoph Seidel , Finn-Ole Paulsen
{"title":"Real-World Data on Outcome of Burkitt Lymphoma and Burkitt Leukemia Treated According to the GMALL B-ALL/NHL 2002 Protocol: A Tertiary Center Experience","authors":"Ricardo Kosch , Christoph Schaefers , Christian Frenzel, Walter Fiedler, Katja Weisel, Carsten Bokemeyer, Christoph Seidel , Finn-Ole Paulsen","doi":"10.1016/j.clml.2025.03.001","DOIUrl":"10.1016/j.clml.2025.03.001","url":null,"abstract":"<div><h3>Introduction</h3><div>Burkitt lymphoma (BL) or leukemia represents a highly aggressive B-cell malignancy requiring intense therapy. Although various different protocols have emerged, few real-world data exist for the GMALL B-ALL/NHL 2002 regimen. Comparative analyses remain challenging because of the disease's rarity.</div></div><div><h3>Methods</h3><div>This retrospective study examined the overall response rate (ORR), progression-free survival (PFS), overall survival (OS), and toxicity in adult patients (pts) with histologically confirmed BL who received the GMALL protocol at the University Medical Center Hamburg-Eppendorf (2012-2022). Statistical analysis identified prognostic factors affecting outcomes.</div></div><div><h3>Results</h3><div>A total of 48 pts (median age 51.5 years, range 22-78) were included. Most presented with Ann Arbor stage IV (68.7%) and high-risk disease by the BL-IPI (54.1%). Burkitt leukemia was present in 27.1%, HIV positivity in 27%, and CNS involvement in 18%. The ORR was 81.6% (73.5% complete response, 8.1% partial response). After a median follow-up of 38 months (range 0-103), the median PFS and OS were not reached; 3-year rates for both PFS and OS were 73%. While not confirmed in multivariate analysis, Burkitt leukemia (HR 3.86, <em>P</em> = .021) and any bone marrow involvement (HR 3.80, <em>P</em> = .049) emerged as adverse prognostic indicators. Severe mucositis (grade ≥ 3) was common (91.7%), and 87.5% experienced at least one febrile neutropenia episode. No grade 5 treatment-related toxicity was observed.</div></div><div><h3>Conclusion</h3><div>These real-world findings underscore the efficacy of the GMALL protocol in BL management, with outcomes comparable to established regimens but coupled with high toxicity rates. Further comparative trials are essential to define the optimal therapeutic strategy.</div></div>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":"25 7","pages":"Pages e533-e540.e2"},"PeriodicalIF":2.7,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143742452","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Outcomes of Newly Diagnosed Multiple Myeloma Patients Requiring Dialysis","authors":"Despina Fotiou , Foteini Theodorakakou , Eirini Solia , Vasiliki Spiliopoulou , Ioannis Ntanasis-Stathopoulos , Panagiotis Malandrakis , Erasmia Psimenou , Nikolaos Kanellias , Maria Roussou , Magdalini Migkou , Evangelos Eleutherakis-Papaiakovou , Angeliki Andrikopoulou , Stavroula Giannouli , Maria Gavriatopoulou , Evangelos Terpos , Efstathios Kastritis , Meletios A. Dimopoulos","doi":"10.1016/j.clml.2025.03.003","DOIUrl":"10.1016/j.clml.2025.03.003","url":null,"abstract":"<div><h3>Introduction</h3><div>Renal impairment (RI) is a common complication in newly diagnosed multiple myeloma (NDMM), with 1-5% of patients presenting with severe RI requiring dialysis, which is associated with significant morbidity and early mortality. Limited real-world data exist on outcomes for these patients.</div></div><div><h3>Aim/Methods</h3><div>We assessed renal response patterns and outcome determinants in 73 consecutive NDMM patients requiring dialysis, treated in a single centre (2010 to 2023).</div></div><div><h3>Results</h3><div>Median age was 69 years; 52% had high-risk cytogenetics. All patients received bortezomib-based induction therapy (19% doublets, 71% triplets, 10% quadruplets; 12% anti-CD38 antibodies). Median follow-up was 37.2 months. Dialysis independence was achieved by 31 patients (42.5%) after a median of 52 days (range 3-247). Dialysis independence was associated with improved survival (median 36 vs. 13.3 months, <em>P</em> = .085) and lower early mortality (3.2% vs. 14.3%, <em>P</em> = .15). Factors associated with independence from dialysis were younger age) OR 0.92, <em>P</em> = .003), hypercalcemia (OR 1.43, <em>P</em> = .013) and hematologic response (≥ PR) at 1 month (OR 3.7, <em>P</em> = .015). In multivariate analysis, younger age (<em>P</em> = .012, OR 0.93) and hematologic response (≥ PR) at 1 month (<em>P</em> = .014, OR 4.94) were independent predictors of dialysis independence. Depth of hematologic response (≥ VGPR) significantly impacted renal recovery (OR 4.0, <em>P</em> = .020). High-risk cytogenetics independently predicted poor outcomes (HR 3.67, <em>P</em> = .003).</div></div><div><h3>Conclusion</h3><div>Dialysis independence is achievable in 42.5% of NDMM patients without special filters in the era of bortezomib-based regimens, with significant impact on outcome. Outcomes remain poor overall for patients who are dialysis-dependent at diagnosis and further evaluation of quadruplet regimens with anti-CD38 antibodies is needed.</div></div>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":"25 7","pages":"Pages 532-538"},"PeriodicalIF":2.7,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691372","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Disease Phenotype Significantly Influences the Outcome After Discontinuation of Ruxolitinib in Chronic Phase Myelofibrosis","authors":"Francesca Palandri , Massimo Breccia , Erika Morsia , Elena M. Elli , Giulia Benevolo , Mario Tiribelli , Eloise Beggiato , Mirko Farina , Giovanni Caocci , Novella Pugliese , Alessia Tieghi , Monica Crugnola , Gianni Binotto , Francesco Cavazzini , Elisabetta Abruzzese , Alessandro Isidori , Alessandra Dedola , Alessandra Iurlo , Roberto M. Lemoli , Daniela Cilloni , Filippo Branzanti","doi":"10.1016/j.clml.2025.02.015","DOIUrl":"10.1016/j.clml.2025.02.015","url":null,"abstract":"<div><h3>Introduction</h3><div>In patients with myelofibrosis (MF), overall survival (OS) after ruxolitinib discontinuation is poor, with leukemic transformation, clonal evolution and thrombocytopenia as the main factors worsening prognosis.</div></div><div><h3>Patients and Methods</h3><div>To assess the impact of disease phenotype on outcome after ruxolitinib discontinuation in chronic phase patients, we performed a sub-analysis of the “RUX-MF” study (NCT06516406), which now includes 1055 MF patients who received ruxolitinib in a real-life context.</div></div><div><h3>Results</h3><div>After a median follow-up of 3.3 years, 397 patients discontinued ruxolitinib therapy while in chronic phase. At treatment end, 208 patients (52.4%) had a severely cytopenic phenotype (defined as platelets < 100 × 10<sup>9</sup>/L and/or hemoglobin < 8 g/dL); among the remaining myeloproliferative 189 patients, 97 had no cytopenia (51.3%) and 92 (48.7%) had mild anemia only (hemoglobin between 8 and 10 g/dL). Overall, 175 patients (44.1%) had a large splenomegaly (palpable at ≥ 10 cm below costal margin).</div><div>After ruxolitinib discontinuation, 3-year OS was 33.4% in severely cytopenic and 54.4% in myeloproliferative patients (<em>P</em> < .001); this was confirmed after adjustment for risk categories. Noncytopenic and mildly anemic patients had comparable OS (<em>P</em> = .73). Patients with large splenomegaly had significantly poorer OS compared to nonsplenomegalic patients (OS: 33.5% vs. 51.6% <em>P</em> = .01). Large splenomegaly confirmed its negative prognostic impact on OS of patients with myeloproliferative MF (60.7% vs. 44.5%, <em>P</em> = .05). In patients with severe cytopenia, the presence of a large splenomegaly did not influence OS (41.7% vs. 26.1%, <em>P</em> = .26).</div></div><div><h3>Conclusions</h3><div>Cytopenic phenotype and large splenomegaly in myeloproliferative MF are key prognostic determinants of outcome after ruxolitinib discontinuation.</div></div>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":"25 7","pages":"Pages e524-e532.e3"},"PeriodicalIF":2.7,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143708889","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Aurore Perrot, Fanny Raguideau, Helene Denis, Martin Prodel, Matthieu Javelot, Marie Pierres, Cyrille Touzeau
{"title":"Survival, Years of Life Lost and Attrition Rates in Multiple Myeloma Patients in France.","authors":"Aurore Perrot, Fanny Raguideau, Helene Denis, Martin Prodel, Matthieu Javelot, Marie Pierres, Cyrille Touzeau","doi":"10.1016/j.clml.2025.03.004","DOIUrl":"https://doi.org/10.1016/j.clml.2025.03.004","url":null,"abstract":"<p><strong>Background: </strong>In a context of strong evolution of multiple myeloma (MM) treatment paradigm, real-world data allow a better understandingthe patients' medical needs.</p><p><strong>Methods: </strong>The present analyses from the MYLORD study were designed to provide recent data on MM patients: characteristics, overall survival (OS), years of life lost (YLL) and attrition rates, using the French National Health Insurance Database (SNDS). It is based on a cohort of 33,032 MM patients who initiated a frontline therapy from 2014 to 2021 in France and who were followed until 2021.</p><p><strong>Results: </strong>The 5-year OS rate is estimated at 51.9% (95% CI: 51.2-52.6). This rate is 78.3% (95% CI: 77.1-79.5) for patients with frontline autologous stem cell transplant (L1T) and 43.6% (95% CI: 42.8-44.4) for those without transplant (L1NT). The excess of mortality in MM patients is revealed by 12.3 YLL versus the matched general population and is higher in L1T patients than those L1NT (17 vs. 11 years). The attrition rate is important in MM patients with 38% of patients who did not benefit from at least 2 lines of treatment and is higher in L1NT patients than those in L1T (41% vs. 29%). Also, the majority of L1NT patients did not receive a L2 because of death, whereas it is because of stay in line status for L1T patients.</p><p><strong>Conclusion: </strong>These results emphasize the urgency to treat MM patients with the best and tailored treatment strategy available, for all MM patients beneficiating or not from a transplant in frontline therapy. Further studies will be needed to assess the impact of new therapeutic options in real life.</p>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143742455","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Chemotherapy Trends in Acute Myeloid Leukemia: 2004 to 2020.","authors":"Aditya Ravindra, Bradley Loeffler, Luna Acharya, Avantika Pyakuryal, Vijaya Raj Bhatt, Prajwal Dhakal","doi":"10.1016/j.clml.2025.02.014","DOIUrl":"https://doi.org/10.1016/j.clml.2025.02.014","url":null,"abstract":"<p><strong>Background: </strong>Chemotherapy is crucial for treating acute myeloid leukemia (AML), as it improves survival and quality of life. However, prior studies have shown that many eligible patients in the United States do not receive chemotherapy due to demographic and socioeconomic disparities.</p><p><strong>Patients and methods: </strong>We utilized the National Cancer Database to analyze chemotherapy utilization in 82,755 patients with AML from 2004 to 2020. We examined trends in 2 time periods, 2004 to 2010 and 2011 to 2019, with a separate analysis for 2020 to evaluate the impact of the COVID pandemic on chemotherapy use.</p><p><strong>Results: </strong>Among all patients with AML, 57.1% received multiagent chemotherapy, 20.5% received single-agent chemotherapy, and 22.4% received no chemotherapy. Chemotherapy use rose from 72.9% in 2004 to 81.3% in 2019, then slightly declined to 80.6% in 2020. The odds of receiving chemotherapy increased significantly in 2011 to 2019 compared to 2004 to 2010 based on age (P = .02), race (P < .01), and AML subtype (P = .03). Patients aged 18 to 40 consistently had higher chemotherapy utilization rates, with treatment odds rising across all age groups. While Black patients were less likely than White patients to receive chemotherapy from 2004 to 2010, their odds improved significantly in 2011 to 2019. Despite increased chemotherapy use across all AML subtypes, therapy-related AML consistently showed the lowest odds of treatment. Lower-income patients, those with more co-morbidities, and female patients had reduced chances of receiving chemotherapy, and these inequities remained largely consistent over time.</p><p><strong>Conclusion: </strong>This large database study highlights improved but persistent disparities based on demographic and socioeconomic status, calling for innovative measures to expand chemotherapy use.</p>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143672918","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Arleigh McCurdy , Engin Gul , Donna Reece , Michael P. Chu , Victor H. Jimenez-Zepeda , Martha Louzada , Kevin Song , Hira Mian , Michael Sebag , Darrell White , Julie Stakiw , Anthony Reiman , Debra Bergstrom , Rami Kotb , Muhammad Aslam , Rayan Kaedbey , Christopher P Venner , Jiandong Su , Richard LeBlanc
{"title":"Treatment Patterns and Outcomes of Multiple Myeloma Patients Undergoing Second-Line Therapy: A Canadian Myeloma Research Group (CMRG) Analysis","authors":"Arleigh McCurdy , Engin Gul , Donna Reece , Michael P. Chu , Victor H. Jimenez-Zepeda , Martha Louzada , Kevin Song , Hira Mian , Michael Sebag , Darrell White , Julie Stakiw , Anthony Reiman , Debra Bergstrom , Rami Kotb , Muhammad Aslam , Rayan Kaedbey , Christopher P Venner , Jiandong Su , Richard LeBlanc","doi":"10.1016/j.clml.2025.02.011","DOIUrl":"10.1016/j.clml.2025.02.011","url":null,"abstract":"<div><h3>Introduction</h3><div>First-line treatment in Canadian transplant-eligible MM patients is CyBorD/RVD followed by ASCT and maintenance lenalidomide. In transplant ineligible patients, bortezomib- or lenalidomide-based treatment is used. Relapse options include salvage ASCT or diverse doublet/triplets. Limited data support any one approach. The aim of this study was to assess treatment patterns/outcomes of real-world patients at first relapse.</div></div><div><h3>Methods</h3><div>All patients initiating second-line therapy from January 1/10 to June 30/22 were included. We evaluated ORR, PFS and OS, calculated from the start of second-line.</div></div><div><h3>Results</h3><div>A total of 3569 patients were included, 1638 (45.9%) with prior ASCT and 1931 (54.1%) without. The most used regimens were Rd, DRd, CyBorD, ASCT, RVd and DVd. The highest ORRs were seen with DRd (90%), ASCT (89%), Kd/KCd (79%), RVd (78.1%) and KRd (74%). For drug regimens, median PFS (27.6 months) and OS (75.5 months) were longest for DRd.</div></div><div><h3>Conclusions</h3><div>We evaluated impact of second-line treatment in MM patients. Triplets with an IMiD backbone and carfilzomib combinations had high ORR and durable PFS, with select patients undergoing second ASCT achieving durable responses. Given the expanding use of IMiD, PI and CD38 mAbs frontline, our results highlight the need for modern modalities at first relapse and provide a benchmark for implementation.</div></div>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":"25 7","pages":"Pages e491-e500.e1"},"PeriodicalIF":2.7,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143742463","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"SOHO State of the Art Updates and Next Questions | Latest Advances in the Management of Primary Mediastinal B-Cell Lymphoma.","authors":"Coen J Lap, Kieron Dunleavy","doi":"10.1016/j.clml.2025.02.013","DOIUrl":"https://doi.org/10.1016/j.clml.2025.02.013","url":null,"abstract":"<p><p>Primary mediastinal B-cell lymphoma (PMBCL) is recognized as a distinct clinicopathologic entity that predominantly affects adolescents and young adults (AYA) and is more common in females. Although PMBCL was previously considered to be a subtype of diffuse large B-cell lymphoma, the clinical, histological, and biological characteristics overlap significantly with those of nodular-sclerosing Hodgkin lymphoma (NS-HL). Over recent years, the shared biology of these 2 entities has been highlighted in several studies, and mediastinal gray zone lymphoma, with features intermediate between PMBCL and NS-HL, has been recognized as a unique molecular entity. Although there is a lack of consensus about the optimal therapeutic strategy for patients with newly diagnosed PMCBL, highly curative treatment regimens that obviate the need for mediastinal radiation therapy (RT) are favored by most. Recently, the results from IELSG-37 were presented and demonstrated that patients with a negative PET/CT scan at the completion of treatment do not require consolidative RT. Progress in understanding the biology of PMBCL and its close relationship to NS-HL have helped pave the way for the investigation of novel strategies, including immune checkpoint inhibitors (ICI), CD30-targeting agents and adoptive T-cell approaches. Currently, a clinic trial is ongoing that is evaluating the role of nivolumab with chemo-immunotherapy for the frontline treatment of PMBCL, after ICI have shown robust responses in patients with relapsed and refractory (R/R) PMBCL. In the R/R setting, studies with anti-CD19 CAR-T-cell therapies and treatments with bispecific antibodies have shown good activity.</p>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143691020","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Britney Le , Noha Soror , Hamid D. Ismail , Mohammed Baker , Salman Abu Shetayyah , Catherine G. Chung , Basem M. William
{"title":"Narrowing of the Racial Disparities Gap in Survival of Patients With Mycosis Fungoides: A Longitudinal Analysis of the SEER Database","authors":"Britney Le , Noha Soror , Hamid D. Ismail , Mohammed Baker , Salman Abu Shetayyah , Catherine G. Chung , Basem M. William","doi":"10.1016/j.clml.2025.02.012","DOIUrl":"10.1016/j.clml.2025.02.012","url":null,"abstract":"<div><h3>Background</h3><div>Mycosis fungoides (MF) is the commonest subtype of cutaneous T-cell lymphoma. In the United States, prior studies reported that African Americans (AA) with MF had poor outcomes. Data characterizing differences in racial disparity outcomes over time are limited.</div></div><div><h3>Patients and Methods</h3><div>We collected data from the United States Surveillance, Epidemiology, and End Results (SEER) database to investigate the survival patterns of patients with MF from 1988 to 2011. Cases were divided into 3 cohorts based on the year of diagnosis. Univariable and multivariable analysis were conducted to assess for factors associated with overall survival (OS).</div></div><div><h3>Results</h3><div>2896 cases of MF were detected, with a median follow-up duration of 60 months. The disparity in survival between the years 1988-1995 and 2004-2011 was significant (<em>P</em> = .05). The parameter estimates of the Cox proportional hazards model for the 1988-1995 period (using the 2004-2011 period as a reference) was also significant (<em>P</em> = .024). Patients diagnosed between 1988 and 1995 were 1.4 times more likely to die from the disease than those diagnosed between 2004 and 2011. The survival gap between AA and white patients narrowed in 1996-2003 and 2004-2011 in comparison to 1988-1995. This indicates improvements in the survival of AA patients over time. Conversely, the survival rates of white patients remained relatively stable over time.</div></div><div><h3>Conclusions</h3><div>Our study demonstrates that AA with MF have reduced survival. Despite the persistent pattern of lower survival across all periods, the gap in survival between white and AA seems to be narrowing over time.</div></div>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":"25 7","pages":"Pages e501-e506"},"PeriodicalIF":2.7,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143630175","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"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":"10.1016/j.clml.2025.02.008","url":null,"abstract":"<div><h3>Background</h3><div>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.</div></div><div><h3>Patients and Methods</h3><div>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).</div></div><div><h3>Results</h3><div>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 <em>(P</em> = .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 (<em>P</em> = .016 and .012, respectively).</div></div><div><h3>Conclusion</h3><div>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.</div></div>","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":"25 7","pages":"Pages e466-e473.e1"},"PeriodicalIF":2.7,"publicationDate":"2025-02-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143639422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rami S. Komrokji, Muhammad Ammad-ud-din, Najla H. Al Ali, Zhuoer Xie, Onyee Chan, Andrew T. Kuykendall, Seongseok Yun, Alison R. Walker, Jeffrey Lancet, Eric Padron, David A. Sallman
{"title":"Hematological Response to Frontline Treatment in Lower Risk Myelodysplastic Syndromes (LRMDS) is Associated With Better Overall Survival","authors":"Rami S. Komrokji, Muhammad Ammad-ud-din, Najla H. Al Ali, Zhuoer Xie, Onyee Chan, Andrew T. Kuykendall, Seongseok Yun, Alison R. Walker, Jeffrey Lancet, Eric Padron, David A. Sallman","doi":"10.1016/j.clml.2025.02.010","DOIUrl":"10.1016/j.clml.2025.02.010","url":null,"abstract":"","PeriodicalId":10348,"journal":{"name":"Clinical Lymphoma, Myeloma & Leukemia","volume":"25 7","pages":"Pages e487-e490"},"PeriodicalIF":2.7,"publicationDate":"2025-02-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143669418","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}