[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献

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[Ethics in clinical research: for participant protection and higher-quality research]. 临床研究中的伦理:保护参与者和提高研究质量
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.1271
Satoshi Nishiwaki
{"title":"[Ethics in clinical research: for participant protection and higher-quality research].","authors":"Satoshi Nishiwaki","doi":"10.11406/rinketsu.66.1271","DOIUrl":"https://doi.org/10.11406/rinketsu.66.1271","url":null,"abstract":"<p><p>Ethics in clinical research are indispensable for participant protection and ensuring research quality, and constitute essential knowledge for all healthcare professionals involved in clinical practice. Learning from historical case studies of unethical research, such as the Nazi human experiments and the Tuskegee Syphilis Study has led to progressive development of ethical guidelines like the Nuremberg Code, the Declaration of Helsinki, and the Belmont Report-which presents the three core principles of respect for persons, beneficence, and justice. In Japan, incidents including the Diovan case prompted the enactment of the Clinical Research Act. The 8 requirements set forth by the U.S. National Institutes of Health (social/scientific value, scientific validity, fair subject selection, favorable risk-benefit ratio, independent review, informed consent, and respect for subjects) serve as a key framework for modern research practice. In Japan, regulations such as the Pharmaceuticals and Medical Devices Act/Good Clinical Practice, the Clinical Research Act, and associated guidelines have been established, and require compliance tailored to the specific type of research. The practice of clinical research adhering to ethical principles forms the foundation for the advancement of medicine built on trust.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 9","pages":"1271-1279"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145208754","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}
引用次数: 0
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.134
{"title":"","authors":"","doi":"10.11406/rinketsu.66.134","DOIUrl":"https://doi.org/10.11406/rinketsu.66.134","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"134-135"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569183","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}
引用次数: 0
[Long-term remission achieved with allogeneic hematopoietic stem cell transplantation at first complete remission in a pediatric patient with TCF7::SPI1 fusion-positive T-cell acute lymphoblastic leukemia]. [同种异体造血干细胞移植在TCF7::SPI1融合阳性t细胞急性淋巴细胞白血病患儿首次完全缓解中实现长期缓解]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.49
Mayuko Noguchi, Kai Yamasaki, Sakiko Azuma, Natsumi Kikuchi, Chika Nitani, Keiko Okada, Nobutaka Kiyokawa, Kiyotaka Isobe, Junko Takita, Hiroyuki Fujisaki, Junichi Hara
{"title":"[Long-term remission achieved with allogeneic hematopoietic stem cell transplantation at first complete remission in a pediatric patient with TCF7::SPI1 fusion-positive T-cell acute lymphoblastic leukemia].","authors":"Mayuko Noguchi, Kai Yamasaki, Sakiko Azuma, Natsumi Kikuchi, Chika Nitani, Keiko Okada, Nobutaka Kiyokawa, Kiyotaka Isobe, Junko Takita, Hiroyuki Fujisaki, Junichi Hara","doi":"10.11406/rinketsu.66.49","DOIUrl":"10.11406/rinketsu.66.49","url":null,"abstract":"<p><p>T-cell acute lymphocytic leukemia (T-ALL) with SPI1 fusion, a leukemia subtype first identified in Japan, has a very poor prognosis. A 7-year-old boy was admitted to our hospital with fever, cervical lymphadenopathy, eyelid edema, and purpura. White blood cell count was markedly increased (551,000/µl). Flow cytometric analysis revealed cyCD3, CD1a, CD8, and HLA-DR positive T-ALL, and fusion gene screening identified TCF7::SPI1 fusion. The patient was treated according to the JPLSG ALL-T11 protocol. He responded poorly to prednisolone, but favorably to L-asparaginase. After completion of early intensification therapy, molecular remission was confirmed. However, due to the patient's poor response to prednisolone, and the presence of the SPI1 fusion gene, hematopoietic stem cell transplantation from an HLA-matched unrelated donor was performed in first remission. So far, the patient has been in remission for 36 months from the time of onset. Hematopoietic stem cell transplantation in first remission may be effective treatment for patients with T-ALL and SPI1 fusion.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 1","pages":"49-53"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383991","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}
引用次数: 0
[Treatment outcomes of axicabtagene ciloleucel for relapsed/refractory diffuse large B-cell lymphoma: a retrospective analysis at a single institution]. [阿昔巴他基西鲁塞治疗复发/难治性弥漫性大b细胞淋巴瘤的疗效:单一机构的回顾性分析]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.81
Wataru Kitamura, Nobuharu Fujii, Chihiro Kamoi, Toshiki Terao, Akira Yamamoto, Hiroki Kobayashi, Takumi Kondo, Keisuke Seike, Hideaki Fujiwara, Keiko Fujii, Noboru Asada, Daisuke Ennishi, Yoshinobu Maeda
{"title":"[Treatment outcomes of axicabtagene ciloleucel for relapsed/refractory diffuse large B-cell lymphoma: a retrospective analysis at a single institution].","authors":"Wataru Kitamura, Nobuharu Fujii, Chihiro Kamoi, Toshiki Terao, Akira Yamamoto, Hiroki Kobayashi, Takumi Kondo, Keisuke Seike, Hideaki Fujiwara, Keiko Fujii, Noboru Asada, Daisuke Ennishi, Yoshinobu Maeda","doi":"10.11406/rinketsu.66.81","DOIUrl":"10.11406/rinketsu.66.81","url":null,"abstract":"<p><p>The advent of anti-CD19 chimeric antigen receptor-T cell therapy has dramatically changed the treatment strategy for relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL). Three products are recently available in Japan, but to the best of our knowledge, real-world data are only available for tisagenlecleucel. This study was a retrospective analysis of 27 patients who received axicabtagene ciloleucel (axi-cel) for R/R DLBCL at our institution. Cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome occurred in 24 (88.9%) and 8 patients (29.6%), respectively, and corticosteroids were used in 19 patients (70.4%). The median follow-up period was 8.1 months (range, 1.0-23.2), and the 6-month progression-free survival and overall survival rates were 80.2% (95% confidence interval [CI], 58.8-91.3) and 92.0% (95%CI, 71.6-97.9), respectively. Although our study was limited by its small sample size and short follow-up period, it demonstrated that axi-cel was highly effective and safe at our institution.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"81-91"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569247","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}
引用次数: 0
[The Utility of Flow Cytometry Profiling Using CD7 and CADM1 in adult T-cell leukemia/lymphoma]. [利用CD7和CADM1在成人t细胞白血病/淋巴瘤中的流式细胞术分析的应用]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.209
Koji Jimbo
{"title":"[The Utility of Flow Cytometry Profiling Using CD7 and CADM1 in adult T-cell leukemia/lymphoma].","authors":"Koji Jimbo","doi":"10.11406/rinketsu.66.209","DOIUrl":"https://doi.org/10.11406/rinketsu.66.209","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 4","pages":"209-219"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144014191","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}
引用次数: 0
[Disseminated intravascular coagulation associated with sepsis]. [弥散性血管内凝血与败血症相关]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.827
Ema Hashiguchi, Takashi Ito
{"title":"[Disseminated intravascular coagulation associated with sepsis].","authors":"Ema Hashiguchi, Takashi Ito","doi":"10.11406/rinketsu.66.827","DOIUrl":"10.11406/rinketsu.66.827","url":null,"abstract":"<p><p>Sepsis is defined as life-threatening organ dysfunction caused by dysregulated host responses to infection. The prothrombotic/antithrombotic balance within blood vessels can be shifted toward prothrombotic in infectious diseases. This is a physiological defense mechanism intended to contain the disturbance in peripheral tissues and prevent it from reaching the central systems. However, in severe infections where this response does not remain localized, extensive ischemic organ damage leads to sepsis-associated disseminated intravascular coagulation (DIC) with multiple organ failure. In such cases, anticoagulation therapy can alleviate ischemic organ damage. The Japanese Clinical Practice Guidelines for Management of Sepsis and Septic Shock 2024 and the Clinical Practice Guidelines for Management of DIC in Japan 2024 recommend antithrombin or recombinant thrombomodulin for sepsis-associated DIC. Further discussion is needed to determine the optimal candidates and methods for treatment.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 8","pages":"827-832"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144994760","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}
引用次数: 0
[Detection of neutralizing antibody against SARS-CoV-2 in a patient with X-linked agammaglobulinemia receiving immunoglobulin replacement therapy]. [1例接受免疫球蛋白替代治疗的x连锁无球蛋白血症患者的SARS-CoV-2中和抗体检测]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.130
Hiroyasu Kaya, Hideki Tani, Masae Itamochi, Kazunori Oishi
{"title":"[Detection of neutralizing antibody against SARS-CoV-2 in a patient with X-linked agammaglobulinemia receiving immunoglobulin replacement therapy].","authors":"Hiroyasu Kaya, Hideki Tani, Masae Itamochi, Kazunori Oishi","doi":"10.11406/rinketsu.66.130","DOIUrl":"10.11406/rinketsu.66.130","url":null,"abstract":"<p><p>Patients with X-linked agammaglobulinemia (XLA) cannot produce antibodies. In this study, we investigated antibodies against SARS-CoV-2 in a man in his 40s with XLA who had no history of COVID-19 or vaccination against COVID-19. Even though his close contact mother contracted SARS-CoV-2, he had never been infected. We measured N-IgG and S-IgG antibodies, as well as neutralizing activity. Serum antibody titers were higher after administration of immunoglobulin, and the antibody titers of immunoglobulin preparations were high as well. Our data revealed that regular administration of immunoglobulins sufficiently increases virus-specific neutralizing antibody activity in patients with XLA.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 2","pages":"130-132"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143569203","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}
引用次数: 0
[Rebalancing therapy for congenital hemophilia]. 先天性血友病的再平衡治疗。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.488
Kagehiro Amano
{"title":"[Rebalancing therapy for congenital hemophilia].","authors":"Kagehiro Amano","doi":"10.11406/rinketsu.66.488","DOIUrl":"10.11406/rinketsu.66.488","url":null,"abstract":"<p><p>Conventional treatments for hemophilia have focused on replacing the missing coagulation factor, but a new treatment concept called rebalancing therapy has recently emerged. Rebalancing therapy corrects bleeding tendency by adjusting the balance between coagulation and anticoagulation, and specifically targets TFPI, AT and APC. The anti-TFPI agents concizumab and marstacimab are administered subcutaneously and have been approved for use in Japan. They are monoclonal antibodies that target the K2 domain of TFPI, and improve hemostatic function by inhibiting the binding of TFPI to FXa and TF/FVIIa complexes. The siRNA drug fitusiran is used as an anti-AT agent that reduces the synthesis of AT, and SerpinPC as an anti-APC agent that specifically inhibits APC. This article will outline the concept of rebalancing therapy and the results of clinical trials, as well as precautions and potential issues during treatment.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"488-494"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577218","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}
引用次数: 0
[Future treatment strategies for acute myeloid leukemia]. [未来急性髓性白血病的治疗策略]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.590
Yuho Najima
{"title":"[Future treatment strategies for acute myeloid leukemia].","authors":"Yuho Najima","doi":"10.11406/rinketsu.66.590","DOIUrl":"10.11406/rinketsu.66.590","url":null,"abstract":"<p><p>Acute myeloid leukemia (AML) is becoming more prevalent as the Japanese population ages, highlighting the growing importance of individualized treatment based on age, comorbidities, and genetic abnormalities. This review outlines the significant transformation in AML management after the introduction of azacitidine plus venetoclax, FLT3 inhibitors, and CPX-351. It also discusses HEM-SIGHT, a next-generation sequencing panel developed in Japan that became covered by Japanese national health insurance in 2025, enabling a molecularly stratified approach to diagnosis. Despite advances in treatment, several subtypes including TP53-mutated and MECOM-overexpressing AML remain highly refractory, even with allogeneic stem cell transplantation and targeted therapies. These high-risk entities pose ongoing therapeutic challenges. The current paradigm in AML treatment has shifted toward strategic personalization, encompassing molecular abnormality identification, measurable residual disease assessment, and treatment adaptation based on these findings. To achieve wider adoption of precision medicine in clinical practice, Japan must continue strengthening its diagnostic systems, streamlining genomic testing in routine practice, and integrating these strategies with novel therapeutic development.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 7","pages":"590-596"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796380","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}
引用次数: 0
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.397
{"title":"","authors":"","doi":"10.11406/rinketsu.66.397","DOIUrl":"https://doi.org/10.11406/rinketsu.66.397","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"397-398"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227922","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}
引用次数: 0
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