{"title":"[Clinical features of three cases with primary bone marrow lymphoma].","authors":"Hidetaka Nakagaki, Takahiro Shima, Reiko Yoneda, Masayasu Hayashi, Sae Utsumi, Seiya Hirakawa, Chiaki Kubara, Ken Takigawa, Akihisa Yoshino, Mariko Minami, Yayoi Matsuo, Takuro Kuriyama, Shuichi Taniguchi, Tetsuya Eto","doi":"10.11406/rinketsu.66.42","DOIUrl":"10.11406/rinketsu.66.42","url":null,"abstract":"<p><p>Primary bone marrow lymphoma (PBML) is a malignant lymphoma characterized by proliferation of lymphoma cells exclusively in the bone marrow without lymphadenopathy. Despite the dismal prognosis of PBML, it is a very rare lymphoma with limited evidence concerning its pathophysiology, making accumulation of cases important. We herein report three cases of PBML at our institution. The first patient was an 80-year-old man who presented with hemophagocytic syndrome and pancytopenia at admission, and died of septic shock during initial chemotherapy. The second patient was a 64-year-old man who achieved complete remission with intensive chemotherapies, but relapsed shortly after completing the final chemotherapy course. The third patient was a 66-year-old woman who underwent chemotherapies and allogeneic hematopoietic stem cell transplantation, only to relapse shortly after transplantation. Although early intervention with chemotherapy is essential for PBML treatment, diagnosis of PBML is very challenging due to the absence of lymph node involvement. Moreover, treatment outcomes of existing chemotherapy and transplantation therapies for PBML are still poor. Further accumulation of cases and development of new treatment strategies are desirable.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"42-48"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384276","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Pharmacogenomics in leukemia treatment].","authors":"Motohiro Kato","doi":"10.11406/rinketsu.66.504","DOIUrl":"10.11406/rinketsu.66.504","url":null,"abstract":"<p><p>Recent advances in molecular genetic research, driven by the development of genomic analysis technologies, have significantly improved treatment outcomes for leukemia. In recent years, mounting evidence indicates that germline genetic background influences drug sensitivity and the risk of adverse effects, underscoring the growing importance of personalized treatment strategies. In particular, the NUDT15 polymorphism, which determines sensitivity to 6-mercaptopurine, has garnered significant attention. Notably, a low-activity variant of this polymorphism, prevalent in Asian countries, has been shown to substantially increase the risk of bone marrow suppression and other adverse effects. Pre-treatment analysis of the NUDT15 polymorphism has demonstrated utility in dose adjustment, helping to mitigate the risk of treatment-related toxicities. Studies have also explored the relationship between genetic background and late complications of leukemia treatment. Optimization of therapeutic strategies based on pharmacogenetic insights holds promise for minimizing complications while maximizing treatment efficacy for each individual patient.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"504-508"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577215","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"","authors":"","doi":"10.11406/rinketsu.66.860","DOIUrl":"https://doi.org/10.11406/rinketsu.66.860","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 8","pages":"860-872"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144994711","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Novel treatment strategies for lower-risk myelodysplastic neoplasms].","authors":"Yasuyoshi Morita","doi":"10.11406/rinketsu.66.581","DOIUrl":"10.11406/rinketsu.66.581","url":null,"abstract":"<p><p>The goal of treatment for lower-risk myelodysplastic neoplasms (MDS) is to improve cytopenia, and therapeutic options should include agents with few adverse events. Since anemia is the main symptom of this disease, red blood cell transfusions, darbepoetin, anabolic steroids, and immunosuppressive agents are considered when the disease is symptomatic, but with the advent of the novel anti-anemic agent luspatercept, there is growing debate regarding the timing of therapeutic intervention and the hemoglobin (Hb) levels at which red blood cell transfusion should be considered in this disease. As a result, there is a growing debate among institutions regarding these issues. It has become clear that there are slight differences in the criteria for initiation of transfusion and the timing of therapeutic intervention with anti-anemic agents at different centers. In this review, we will discuss the extent to which therapeutic intervention for lower-risk MDS improves the quality of life (QOL) of patients and how to improve the efficiency of limited healthcare resources, including an overview of ongoing clinical trials.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 7","pages":"581-589"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796386","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Prevention and treatment of post-transplant relapse in adult T-cell leukemia-lymphoma].","authors":"Ayumu Ito","doi":"10.11406/rinketsu.66.612","DOIUrl":"https://doi.org/10.11406/rinketsu.66.612","url":null,"abstract":"<p><p>Aggressive adult T-cell leukemia-lymphoma (ATL) is a rare T-cell malignancy with a poor prognosis. Allogeneic hematopoietic cell transplantation (allo-HCT) is a curative therapy, but the long-term survival rate is approximately 40%, which is poor compared to other hematopoietic tumors. The most common cause of death after allo-HCT is ATL relapse. Approximately 40% of patients experience a relapse following allo-HCT, and the prognosis after relapse remains poor. In recent years, various strategies have been attempted to prevent and treat post-transplant ATL relapse. Cord blood and HLA haplo-identical related donors are widely used as alternative donors to Japan Marrow Donor Program donors for transplantation in remission soon after diagnosis, and novel drugs for ATL are used as salvage therapy before or after allo-HCT. Monitoring of measurable residual disease with flow cytometry has also been attempted for early detection of post-transplant relapse. Nevertheless, there is still insufficient evidence for the prevention and treatment of post-transplant relapse of ATL. Given the limited experience at individual hospitals and the increasing population of HTLV-1 carriers throughout the country, it is desirable to build evidence based on treatment experience and analysis of clinical specimens from hospitals throughout Japan.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 7","pages":"612-620"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"","authors":"","doi":"10.11406/rinketsu.66.718","DOIUrl":"https://doi.org/10.11406/rinketsu.66.718","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 7","pages":"718-726"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796354","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Development and performance evaluation of a comprehensive genomic profiling assay for hematological malignancies].","authors":"Yasunori Kogure, Yuji Oshikawa-Kumade, Suguru Fukuhara, Kenichi Chiba, Ai Okada, Motohiro Kato, Yasuhito Nannya, Mamiko Sakata-Yanagimoto, Masaki Ri, Takahiro Maeda, Hideki Muramatsu, Takayuki Ishikawa, Masashi Sanada, Yasunori Ueda, Daisuke Ennishi, Ryo Higashiyama, Daisuke Koga, Yuichi Shiraishi, Koji Izutsu, Keisuke Kataoka","doi":"10.11406/rinketsu.66.693","DOIUrl":"https://doi.org/10.11406/rinketsu.66.693","url":null,"abstract":"<p><p>Evaluation of genetic alterations is important for diagnosis, treatment selection, and prognostic assessment in hematological malignancies, but no comprehensive genomic profiling (CGP) assays for hematological malignancies have been approved for manufacturing and marketing in Japan. We have developed a CGP assay for the analysis of genetic alterations (specifically, mutations, structural variations, and fusion genes) in 452 genes, along with a corresponding analysis and reporting system that runs on Amazon Web Services. This CGP assay, named HemeSight®, successfully detected genetic alterations with an allele frequency of 5-10% in limit of detection testing using non-clinical samples. In testing using human clinical samples, it showed high positive percent agreement (94.4% for mutations, 94.7% for IGH rearrangements [structural variations], and 100% for fusion genes) compared with established clinical tests.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 7","pages":"693-704"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144982048","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Mechanisms of resistance to the allosteric BCR::ABL1 inhibitor asciminib].","authors":"Seiichi Okabe","doi":"10.11406/rinketsu.66.1033","DOIUrl":"https://doi.org/10.11406/rinketsu.66.1033","url":null,"abstract":"<p><p>Asciminib is a first-in-class allosteric inhibitor that specifically targets the myristoyl pocket of BCR::ABL1 and has shown efficacy in patients with chronic myeloid leukemia (CML) who are resistant or intolerant to prior tyrosine kinase inhibitors (TKIs). Despite its unique mechanism of action, several resistance mechanisms to asciminib have been identified. BCR::ABL1 kinase domain mutations, including A337V, C464W, and compound mutations involving T315I, can interfere with asciminib binding or allosteric regulation. Additionally, BCR::ABL1 transcript variants lacking the SH3 domain, such as e13a3 and e14a3, exhibit primary resistance by disrupting the autoinhibited conformation required for asciminib activity. Non-BCR::ABL1 mechanisms that also contribute to resistance include overexpression of efflux transporters such as ABCG2 and P-glycoprotein, which reduce intracellular drug accumulation. Moreover, novel insertion mutations like p.I293_K294insSLLRD have been shown to impair the allosteric inhibition of ABL1. Combination therapies with ponatinib or other agents, as well as newer TKIs like olverembatinib, have demonstrated potential in overcoming resistance in preclinical and clinical models. Understanding these diverse resistance mechanisms is critical for optimizing asciminib-based treatment strategies and guiding the development of effective combination therapies for patients with resistant CML.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 9","pages":"1033-1041"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208734","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Treatment of pediatric chronic myeloid leukemia].","authors":"Chikako Tono","doi":"10.11406/rinketsu.66.1241","DOIUrl":"https://doi.org/10.11406/rinketsu.66.1241","url":null,"abstract":"<p><p>Treatment strategies for chronic myeloid leukemia (CML) have changed significantly with the development of new tyrosine kinase inhibitors (TKIs). Due to its extreme rarity, pediatric CML has historically been managed based on evidence in adult patients. However, as the unique biological and clinical characteristics of pediatric CML have become increasingly apparent, the need for pediatric-specific treatment guidelines is now widely recognized. This review outlines the treatment of pediatric CML as of 2025, with a focus on clinical trial results from Japan and the latest consensus guidelines issued by the International Pediatric CML Working Group.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 9","pages":"1241-1251"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208768","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Onco-cardiology in hematological malignancy: from the perspective of cardiology].","authors":"Hiroshi Akazawa","doi":"10.11406/rinketsu.66.1094","DOIUrl":"https://doi.org/10.11406/rinketsu.66.1094","url":null,"abstract":"<p><p>Recent advances in cancer therapy have improved the long-term outcomes of cancer patients, increasing the importance of managing cardiovascular complications arising not only from cancer itself but also from chemotherapy, radiation therapy, and immunotherapy. In the field of hematology, there are serious concerns about cardiovascular complications of various chemotherapeutic agents such as anthracyclines, immunomodulatory drugs, BCR-ABL tyrosine kinase inhibitors, proteasome inhibitors, and immune checkpoint inhibitors. Chemotherapy has a wide variety of effects on the cardiovascular system, and the molecular mechanisms underlying the cardiovascular toxicities of individual molecularly targeted agents remain to be precisely defined. This \"Onco-Cardiology\" approach is expected to enhance interdisciplinary collaboration between oncology/hematology and cardiology specialists across clinical practice, research, and education to protect cancer patients and survivors from cardiovascular complications.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 9","pages":"1094-1099"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208721","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}