{"title":"New classifications of B-cell neoplasms: a comparison of 5th WHO and International Consensus classifications.","authors":"Kennosuke Karube, Akira Satou, Seiichi Kato","doi":"10.1007/s12185-024-03781-5","DOIUrl":"10.1007/s12185-024-03781-5","url":null,"abstract":"<p><p>In 2024, the World Health Organization (WHO) launched a new classification of lymphoid neoplasms, a revision of the previously used Revised 4th Edition of their classification (WHO-4R). However, this means that two classifications are now in simultaneous use: the 5th Edition of the WHO classification (WHO-5) and the International Consensus Classification (ICC). Instead of a comprehensive review of each disease entity, as already described elsewhere, this review focuses on revisions made in both the WHO-5 and ICC from WHO-4R and discrepancies between them regarding B-cell neoplasms. Similarities include cutaneous marginal zone lymphoma, cold agglutinin disease, non-primary effusion lymphoma-type effusion-based lymphoma, and gray zone lymphoma. Differences include plasma cell neoplasms, high-grade B-cell lymphoma (double hit lymphoma), follicular lymphoma, LPD with immune deficiency and dysregulation, extranodal large B-cell lymphoma, transformations of indolent B-cell lymphomas, and diffuse large B-cell lymphoma, not otherwise specified. Understanding the similarities and differences between the two latest classifications will aid daily diagnostic practice and future research on lymphoid neoplasms.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"331-341"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141156857","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":"Recent advances in understanding the biology of follicular lymphoma.","authors":"Momoko Nishikori","doi":"10.1007/s12185-024-03764-6","DOIUrl":"10.1007/s12185-024-03764-6","url":null,"abstract":"<p><p>Follicular lymphoma (FL), the most common indolent B-cell lymphoma, develops over decades before manifesting as overt disease. BCL2 overexpression by t(14;18) confers a survival advantage to B cells during the germinal center reaction, and abnormalities in epigenetic modifier genes lead to desynchronization of gene expression changes in germinal center B cells. Studies in mouse models have shown that BCL2 overexpression and epigenetic deregulation in B cells cooperatively promote lymphomagenesis. The immune microenvironment also plays an essential role in the biology of FL, and many molecular prognostic indicators based on the immune microenvironment have been proposed. However, high-risk gene signatures do not appear to be consistent between patients receiving different chemotherapies. FL cells frequently carry N-linked glycosylation motifs within the immunoglobulin gene, leading to chronic activation of the B-cell receptor (BCR). Recent evidence suggests that this chronic BCR signaling drives FL polarization toward a dark-zone phenotype and promotes clonal evolution. Since both epigenetic and post-transcriptional modifications of B cells have been implicated in the early stage of FL development, it may be possible to use novel non-chemotherapeutic approaches that interfere with the immunobiology in treatment or early prevention of FL.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"326-330"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140305538","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":"Pseudo-Chediak-Higashi inclusions and Auer rods in a case of therapy-related acute monocytic leukemia.","authors":"Radu Chiriac","doi":"10.1007/s12185-024-03910-0","DOIUrl":"10.1007/s12185-024-03910-0","url":null,"abstract":"","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"289-290"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909646","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":"Temporal changes in corticosteroid dose during ibrutinib treatment in patients with cGVHD and pulmonary involvement.","authors":"Masako Toyosaki, Shinichiro Machida, Daisuke Tomizawa, Masaya Okada, Masashi Sawa, Yasunori Ueda, Ai Omi, Yosuke Koroki, Takanori Teshima","doi":"10.1007/s12185-024-03882-1","DOIUrl":"10.1007/s12185-024-03882-1","url":null,"abstract":"<p><p>The GVH3001 study assessed the efficacy and safety of ibrutinib in Japanese patients with steroid-dependent or -refractory chronic graft-versus-host disease (cGVHD). However, the effects of ibrutinib on lung function and reduction in corticosteroid dose, which is a measurable factor associated with improved quality of life, could not be adequately assessed in patients who initially presented with lung involvement. This post hoc analysis aimed to evaluate temporal changes in daily corticosteroid dose, as well as effectiveness outcomes based on lung function and symptom burden (percent predicted forced expiratory volume in 1 s [%FEV<sub>1</sub>] and Lee cGVHD Symptom Scale lung subscale score, respectively) in the subgroup of patients with cGVHD who had lung involvement at baseline. Seven of the 19 patients in the GVH3001 study had lung involvement at baseline. The daily corticosteroid dose for cGVHD decreased in five of these patients, and %FEV<sub>1</sub> remained relatively stable in two patients but increased to > 80% in one patient. Lee cGVHD Symptom Scale scores were relatively stable throughout the study in patients with lung involvement. Ibrutinib may allow corticosteroid dose reduction without worsening lung function or increasing symptom burden in previously treated patients with cGVHD and associated lung involvement.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"388-396"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11861523/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142800668","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Current treatment approach and future perspectives in B cell lymphoma.","authors":"Nobuhiko Yamauchi, Dai Maruyama","doi":"10.1007/s12185-024-03879-w","DOIUrl":"10.1007/s12185-024-03879-w","url":null,"abstract":"<p><p>Diffuse large B cell lymphoma (DLBCL) and follicular lymphoma (FL) represent the two major subtypes of mature B cell lymphoma. A deeper understanding of tumor biology, as well as molecular classification characterized by targetable gene alterations, and the introduction of novel treatment options, including targeted drugs (e.g., antibody-drug conjugates and small molecules [e.g., Bruton tyrosine kinase inhibitor]) and immune therapies (e.g., chimeric antigen receptor [CAR] T cell therapy and bispecific antibody [BsAb]), has changed the treatment paradigms for DLBCL and FL. In clinical practice, however, treatment regimens are determined mainly based on prior treatment history, duration of response after previous treatment, patient age, and patient frailty because there have been few randomized trials to inform treatment selection for patients with relapsed or refractory disease and because there is no single prognostic index that guides suitable treatment for each patient. In this review, we summarize the treatment options for DLBCL and FL and discuss the treatment strategies for these two subtypes. We also discuss future perspectives for the treatment of these subtypes.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"342-355"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142686904","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":"Graft CD8<sup>+</sup> T cells for improving event-free survival after T cell-replete haploidentical stem cell transplantation in children with hematological malignancies.","authors":"Nobuhisa Takahashi, Kazuhiro Mochizuki, Atsushi Kikuta, Yoshihiro Ohara, Shingo Kudo, Kazuhiko Ikeda, Hitoshi Ohto, Hideki Sano","doi":"10.1007/s12185-024-03900-2","DOIUrl":"10.1007/s12185-024-03900-2","url":null,"abstract":"<p><p>T cell-replete haploidentical hematopoietic stem cell transplantation (TCR-haplo-HSCT) is a potentially curative therapy for pediatric intractable hematological malignancies due to its graft-versus-leukemia efficacy. This single-center cohort study examined the effects of graft composition (T cell type and dose) on pediatric TCR-haplo-HSCT outcomes in 32 children with relapsed/intractable hematological malignancies. Graft T cell composition was classified using flow cytometry. High graft CD8<sup>+</sup> T cell doses reduced disease relapse and improved overall survival and event-free survival, but did not increase transplant-related mortality and the incidence of grade III/IV acute graft-versus-host disease. Doses of grafted CD3<sup>+</sup>, CD4<sup>+</sup>, and CD34<sup>+</sup> T cells did not affect patient outcomes. Children with differing event-free survival times were divided by a graft CD8<sup>+</sup> T cell dose cut-off of 2.03 × 10<sup>8</sup> kg<sup>-1</sup>. These findings revealed that grafted CD8<sup>+</sup> T cells improved the graft-versus-leukemia effect of pediatric TCR-haplo-HSCT without increasing the risk of transplant-related mortality.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"403-410"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142800665","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":"Treatment trends and risks of corticosteroid use in adult primary immune thrombocytopenia: a claims database study in Japan.","authors":"Hirokazu Kashiwagi, Isao Miura, Naohiko Terasawa, Ken-Ichi Iwayama, Yuka Furukawa, Makoto Kanenishi","doi":"10.1007/s12185-024-03897-8","DOIUrl":"10.1007/s12185-024-03897-8","url":null,"abstract":"<p><p>Recent trends in the treatment of primary immune thrombocytopenia (ITP) were investigated using a claims database that included data from 16,161 Japanese patients with ITP collected from April 2014 to August 2022. Of the 4144 adult patients analyzed, 1276 received corticosteroids. The mean and median durations of corticosteroid use were 115.31 and 41 days, respectively. The time to withdrawal of corticosteroids was significantly shorter in 2020 to 2021 than in 2015 to 2019. Additionally, the number of prescriptions for thrombopoietin receptor agonists increased from 2015 to 2021 and exceeded that of corticosteroids in 2021. While these results suggest a trend towards reduction in corticosteroid use in real-world settings in Japan, 12.00% of patients received a corticosteroid dose of ≥ 10 mg/day at Week 12. Furthermore, 23.05% of patients continued to receive corticosteroids at Week 24, indicating that some patients were still receiving long-term corticosteroid treatment. The risk of adverse outcomes was significantly associated with corticosteroid use. In conclusion, new treatment options may lead to more sophisticated ITP management with less corticosteroid use, although further research and reconsideration of clinical practice guidelines is needed.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"363-377"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11861122/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818123","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Successful treatment of type I cryoglobulinemia with a combination of carfilzomib, cyclophosphamide, and dexamethasone: a case report and literature review.","authors":"Yohei Yasuda, Hiroaki Maki, Arika Shimura, Akira Honda, Yosuke Masamoto, Mineo Kurokawa","doi":"10.1007/s12185-024-03911-z","DOIUrl":"10.1007/s12185-024-03911-z","url":null,"abstract":"<p><p>Type I cryoglobulinemia is typically associated with hematological malignancies such as B-cell lymphomas and plasma cell neoplasms. Its treatment basically targets underlying hematological malignancies, and the prognosis remains unsatisfactory. Despite several reports of type I cryoglobulinemia treated with bortezomib-based regimens, little is available on the treatment of bortezomib-resistant cases. We report a case of severe type I cryoglobulinemia associated with plasma cell neoplasm, refractory to bortezomib and daratumumab, which was successfully managed with a combination of carfilzomib, cyclophosphamide, and dexamethasone (KCd therapy). No sign of relapse has been seen for more than 3 years with maintenance therapy with ongoing carfilzomib. This case highlights the potential efficacy of carfilzomib-based regimens in bortezomib-resistant type I cryoglobulinemia, offering a promising option for cases refractory to conventional treatments.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"411-415"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11861407/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143188962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Long-term safety and efficacy of fostamatinib in Japanese patients with primary immune thrombocytopenia.","authors":"Masataka Kuwana, Yoshiaki Tomiyama","doi":"10.1007/s12185-025-03924-2","DOIUrl":"10.1007/s12185-025-03924-2","url":null,"abstract":"<p><p>Fostamatinib had superior efficacy to a placebo and acceptable safety profiles for at least 1 year in a phase 3 study of Japanese patients with primary immune thrombocytopenia. Here, we report the 3-year safety and efficacy of fostamatinib in that study. Data from 33 patients who received at least one dose of fostamatinib were analyzed. A platelet response > 50,000/µL (at two consecutive visits at least 28 days apart while receiving fostamatinib) was achieved in 16 patients (48%). The median total duration of a platelet response > 50,000/µL was 589 (range: 106-1003) days. Gastrointestinal disorders, such as diarrhea, hypertension, and hepatic enzyme elevation, were the most common fostamatinib-related adverse events. Most events occurred within 12 weeks of treatment. No thromboembolisms, treatment-related infections, or moderate or severe treatment-related bleeding events were observed. In summary, this extension study of a clinical trial found a sustained platelet response without new safety signals during 3-year treatment with fostamatinib.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"356-362"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11861395/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143052423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Understanding the intrinsic biology of diffuse large B-cell lymphoma: recent advances and future prospects.","authors":"Yusuke Naoi, Daisuke Ennishi","doi":"10.1007/s12185-024-03780-6","DOIUrl":"10.1007/s12185-024-03780-6","url":null,"abstract":"<p><p>Diffuse large B-cell lymphoma (DLBCL) is the most common type of lymphoid tumor, and accounts for approximately 30-40% of non-Hodgkin lymphomas. Although the prognosis has significantly improved with the advent of rituximab combination chemotherapy in the early 2000s, recurrence still occurs in about 40% of cases. Even though chemotherapy with increased dose-intensity is used in recurrent cases, the prognosis of such patients remains poor. Thus, the development of personalized medicine, including molecular-targeted drugs, is required to improve the prognosis of DLBCL patients, and further understanding of the molecular pathogenesis of DLBCL is essential for this purpose. With recent advances in genetic analysis technology, unknown genetic abnormalities and gene expression patterns have been discovered, and based on these discoveries, progress is being made in elucidating and subdividing molecular pathologies. This article summarizes recent findings regarding molecular pathogenesis in DLBCL using transcriptome and genomics technologies, and outlines the path to personalized medicine.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"321-325"},"PeriodicalIF":1.7,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140897569","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}