Seminars in hematology最新文献

{"title":"Insights from the 12th International Workshop on Waldenstrom's Macroglobulinemia","authors":"Steven P. Treon MD, PhD , Christopher J. Patterson MS , Jeffrey Matous , Christian Buske","doi":"10.1053/j.seminhematol.2025.05.001","DOIUrl":"10.1053/j.seminhematol.2025.05.001","url":null,"abstract":"","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 2","pages":"Pages 71-75"},"PeriodicalIF":5.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144470362","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Report of Consensus Panel 6 from the 12th International Workshop on Waldenstrom's Macroglobulinemia on Diagnosis and Management of Transformed Waldenstrom's Macroglobulinemia","authors":"Eric Durot ,&nbsp;Jithma P. Abeykoon ,&nbsp;Damien Roos-Weil ,&nbsp;M.J. Kersten ,&nbsp;Charalampia Kyriakou ,&nbsp;David F. Moreno ,&nbsp;Stephen M. Ansell ,&nbsp;Rebecca Auer ,&nbsp;Xinxin Cao ,&nbsp;Roger G. Owen ,&nbsp;Shuhua Yi ,&nbsp;Irene Dogliotti ,&nbsp;Marek Trneny ,&nbsp;Christopher J. Patterson ,&nbsp;Jeffrey V. Matous ,&nbsp;Christian Buske ,&nbsp;Steven P. Treon ,&nbsp;Ranjana Advani","doi":"10.1053/j.seminhematol.2025.04.003","DOIUrl":"10.1053/j.seminhematol.2025.04.003","url":null,"abstract":"<div><div>Histological transformation (HT) in Waldenström’s macroglobulinemia (WM) is a rare complication and despite growing literature in the last years, no consensus recommendations exist. Consensus Panel 6 (CP6) of the 12th International Workshop on Waldenström’s Macroglobulinemia (IWWM-12) was convened to review the current data on transformed WM and make recommendations on its diagnosis and management. The key recommendations from IWWM-12 CP6 included: (1) in case of suspected HT, tissue biopsy is the gold standard for diagnosis; (2) the initial work-up should comprise ¹⁸FDG-PET/CT for the evaluation of disease extent and, for patients with clinical suspicion or for high-risk patients (CNS-IPI, multiple and/or specific extranodal involvements), cerebrospinal fluid examination and brain MRI; (3) standard dose chemoimmunotherapy (CIT) such as R-CHOP (rituximab, cyclophosphamide, doxorubicine, vincristine and prednisone) or R-CHP + polatuzumab vedotin are the preferred front-line regimen; (4) CNS prophylaxis and consolidation with autologous stem cell transplantation (SCT) can be considered according to de novo diffuse large B-cell lymphoma (DLBCL) guidelines; (5) T-cell-engaging therapies (CAR T-cells, bispecific antibodies) should be used in the relapse/refractory setting according to international guidelines for DLBCL and local access to these therapies. Key unanswered questions include the role of <em>TP53</em> abnormalities and <em>CXCR4</em> mutations on the risk of HT, the prognostic role of clonal relationship between WM and HT, the optimal front-line therapy (addition of novel agents to CIT, dose-intensive CIT, consolidation with autologous SCT), and the sequence of T-cell-engaging therapies. International collaboration and consideration of and inclusion in clinical trials is critical to address these issues in a rare patient population.</div></div>","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 2","pages":"Pages 120-125"},"PeriodicalIF":5.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144094712","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Report of Consensus Panel 2 from the 12th International Workshop on the management of Bing-Neel syndrome in patients with Waldenstrom’s Macroglobulinemia","authors":"Shayna Sarosiek ,&nbsp;Anne-Marie L. Becking ,&nbsp;Andrew Branagan ,&nbsp;Simone Ferrero ,&nbsp;Jahanzaib Khwaja ,&nbsp;Eva Kimby ,&nbsp;Damien Roos-Weil ,&nbsp;Naohiro Sekiguchi ,&nbsp;Marek Trneny ,&nbsp;Shuhua Yi ,&nbsp;Christopher J. Patterson ,&nbsp;Christian Buske ,&nbsp;Jeffrey V. Matous ,&nbsp;Steven P. Treon ,&nbsp;Monique C. Minnema","doi":"10.1053/j.seminhematol.2025.04.005","DOIUrl":"10.1053/j.seminhematol.2025.04.005","url":null,"abstract":"<div><div>Consensus panel 2 from the 12th International Workshop on Waldenstrom Macroglobulinemia was tasked with updating the guidelines on the diagnosis and management of patients with Bing-Neel syndrome (BNS). In this panel we have summarized the clinical symptoms that may be present with BNS, discussed the criteria required for diagnosis of BNS, made recommendations for follow-up imaging, and proposed revised guidelines for response assessment in BNS. The key recommendations from the 12th International Workshop on WM (IWWM-12) Consensus panel 2 include: (1) the establishment of zanubrutinib as a standard therapy for treatment of BNS; (2) recommendations on imaging and CSF evaluation during treatment and follow-up of BNS; and (3) revised response criteria in view of new data showing that malignant cells can persist in the CSF of many patients treated with BTK-inhibitors. New categorical response categories proposed include that for a Clinical Complete Response and Progressive Disease.</div></div>","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 2","pages":"Pages 85-89"},"PeriodicalIF":5.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144143461","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Modernizing multiple myeloma clinical trial eligibility to improve equity and inclusivity by hematological parameters","authors":"Lauren Merz ,&nbsp;Monique Hartley-Brown ,&nbsp;Maureen Achebe ,&nbsp;Craig Cole ,&nbsp;Bindu Kanapuru ,&nbsp;Ola Banjo ,&nbsp;George Mulligan ,&nbsp;Katie Wozniak ,&nbsp;Anne Quinn Young ,&nbsp;Hearn Jay Cho","doi":"10.1053/j.seminhematol.2024.10.008","DOIUrl":"10.1053/j.seminhematol.2024.10.008","url":null,"abstract":"<div><div>In the United States, Black people experience multiple myeloma (MM) at a frequency that is more than double that of White people and experience much higher rates of mortality. Despite bearing a disproportionate impact of both MM incidence and mortality, Black patients are significantly underrepresented in most MM clinical trials. This is in part because Black patients experience a higher prevalence of hemoglobinopathies and Duffy-null phenotype, which affect hemoglobin and neutrophil levels, respectively, potentially excluding patients from clinical trials. The Multiple Myeloma Research Foundation (MMRF) has convened a series of Health Equity Summits that include a focus on creating inclusive clinical trials for MM. The present paper, an output of the most recent workshop, focuses on the role of laboratory reference ranges as a barrier to clinical trial participation and offers tangible steps to improve the enrollment of a diverse and representative population.</div></div>","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 1","pages":"Pages 38-42"},"PeriodicalIF":5.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142710945","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"outside front cover, PMS 8883 metallic AND 4/C","authors":"","doi":"10.1053/S0037-1963(25)00004-6","DOIUrl":"10.1053/S0037-1963(25)00004-6","url":null,"abstract":"","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 1","pages":"Page CO1"},"PeriodicalIF":5.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143620478","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"FaMMily Affairs: Dissecting inherited contributions to multiple myeloma risk","authors":"Saoirse Bodnar ,&nbsp;Tehilla Brander ,&nbsp;Julie Gold ,&nbsp;Ayuko Iverson ,&nbsp;Alessandro Lagana ,&nbsp;Kenan Onel ,&nbsp;Sundar Jagannath ,&nbsp;Samir Parekh ,&nbsp;Santiago Thibaud","doi":"10.1053/j.seminhematol.2024.11.006","DOIUrl":"10.1053/j.seminhematol.2024.11.006","url":null,"abstract":"<div><div>Etiological links to multiple myeloma (MM) remain poorly understood, though emerging evidence suggests a significant hereditary component. This review integrates current literature on inherited factors contributing to MM risk, synthesizing both epidemiologic and genomic data. We examine familial clustering patterns, assess genome-wide association studies (GWAS) that reveal common genetic variants linked to MM, and explore rare, high-penetrance variants in key susceptibility genes. Additionally, we advocate for routine germline screening in high-risk MM populations, particularly those with a strong family history of cancer, a personal history of cancer, or early-onset disease. By elucidating the inherited influences on MM predisposition, this review seeks to inform future research and refine risk assessment strategies in this population.</div></div>","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 1","pages":"Pages 11-19"},"PeriodicalIF":5.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142897072","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"At the cusp of a cure in Myeloma: Insights into pathogenesis, modeling and therapeutics","authors":"Samir Parekh MD","doi":"10.1053/j.seminhematol.2025.02.002","DOIUrl":"10.1053/j.seminhematol.2025.02.002","url":null,"abstract":"","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 1","pages":"Pages 1-2"},"PeriodicalIF":5.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143620479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of 1q abnormalities in multiple myeloma: Genomic insights, clinical implications, and therapeutic challenges","authors":"Zachary M. Avigan ,&nbsp;Constantine S. Mitsiades ,&nbsp;Alessandro Laganà","doi":"10.1053/j.seminhematol.2024.10.001","DOIUrl":"10.1053/j.seminhematol.2024.10.001","url":null,"abstract":"<div><div>Chromosome 1q copy number variations, collectively termed +1q, are 1 of the most common cytogenetic abnormalities in multiple myeloma. 1q abnormalities are associated with overexpression of a high-risk gene signature promoting cell proliferation, apoptosis resistance, genomic instability, and treatment resistance, and acquisition or expansion of +1q subclones mediate disease development and relapse. While there remains significant controversy as to whether the presence of +1q is itself an independent driver of poor prognosis or is simply a marker of other high-risk features, +1q has recently been incorporated into multiple prognostic scoring models as a new high-risk cytogenetic abnormality. In this review, we present possible underlying genetic mechanisms of high-risk disease in +1q myeloma, implications for subclonal development, its role in modifying the tumor microenvironment, current evidence for clinical significance in newly-diagnosed and relapsed patients, and current controversies in +1q classification and prognostication.</div></div>","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 1","pages":"Pages 20-30"},"PeriodicalIF":5.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142558720","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Melanoma antigen genes (MAGE); novel functional targets in multiple myeloma","authors":"Anna Huo-Chang Mei ,&nbsp;Alessandro Laganà ,&nbsp;Roman Osman ,&nbsp;Hearn Jay Cho","doi":"10.1053/j.seminhematol.2024.10.007","DOIUrl":"10.1053/j.seminhematol.2024.10.007","url":null,"abstract":"<div><div>Melanoma Antigen Genes (MAGE) are expressed in a broad range of cancers, including multiple myeloma. MAGE have been under investigation for more than 3 decades as targets for immune therapy, while in parallel, interrogation of their functions has revealed activities that may be particularly critical in multiple myeloma. MAGE-C1 is expressed in about 75% of newly diagnosed cases and this is maintained through the natural history of the disease. In contrast, MAGE-A3 is expressed in about 35% of newly diagnosed cases, but this increases to more than 75% after relapse. MAGE-A3 expression was associated with poor clinical outcome and resistance to chemotherapy. Translational studies have revealed that MAGE-A3 regulates cell cycling and apoptosis in myeloma cells. Genomic, gene expression, and multiomic studies demonstrate relations with high-risk subgroups of patients. MAGE-A3 mediates these functions through partnership with Kap1 to form a ubiquitin ligase complex. Structural analysis of the interaction between MAGE-A3 and Kap1 gives insight into the biochemical activity and substrate specificity and suggests novel pharmacologic strategies to inhibit them. These studies demonstrating MAGE-A3 oncogenic functions suggest that it may also be a suitable target for small molecule inhibition in multiple myeloma that may be broadly applicable to other cancers that express it.</div></div>","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 1","pages":"Pages 43-49"},"PeriodicalIF":5.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142695903","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Smoldering multiple myeloma: Integrating biology and risk into management","authors":"Roshani Patel ,&nbsp;Elizabeth Hill ,&nbsp;Madhav Dhodapkar","doi":"10.1053/j.seminhematol.2024.10.002","DOIUrl":"10.1053/j.seminhematol.2024.10.002","url":null,"abstract":"<div><div>Smoldering multiple myeloma (SMM) was first described over 40 years ago yet much is still unknown including which patients will ultimately progress to symptomatic multiple myeloma (MM). The genetics of the premalignant clone and the immune microenvironment in which it exists is now well understood to both play a role in disease progression. However, the clinical risk models available to help identify patients at most risk of progression still rely primarily on data reflecting volume of disease rather than underlying biology. While it is of upmost importance to accurately diagnose patients with SMM to avoid over or under treatment, efforts are ongoing to tease out if early intervention is indeed warranted for a subgroup of patients with SMM. This article will review the history and biology of SMM, discuss the utility of existing risk models, and examine the efforts to date which have challenged standard management.</div></div>","PeriodicalId":21684,"journal":{"name":"Seminars in hematology","volume":"62 1","pages":"Pages 3-10"},"PeriodicalIF":5.0,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142740422","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}