[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献_第4页

[CAR-T cell therapy for acute lymphoblastic leukemia: current landscape and novel developments]. CAR-T细胞治疗急性淋巴细胞白血病：现状和新进展。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.1366

Chihaya Imai

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[Advances in the treatment of relapsed or refractory acute lymphoblastic leukemia]. [复发或难治性急性淋巴细胞白血病治疗进展]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.1132

Etsuko Yamazaki

{"title":"[Advances in the treatment of relapsed or refractory acute lymphoblastic leukemia].","authors":"Etsuko Yamazaki","doi":"10.11406/rinketsu.67.1132","DOIUrl":"https://doi.org/10.11406/rinketsu.67.1132","url":null,"abstract":"Relapsed or refractory acute lymphoblastic leukemia remains associated with poor outcomes, with conventional chemotherapy offering limited long-term survival. In recent years, the introduction of immunotherapies, including antibody-based agents and chimeric antigen receptor T-cell (CAR-T) therapy, has improved treatment outcomes; however, optimization of therapeutic strategies remains a critical challenge. The anti-CD22 antibody-drug conjugate inotuzumab ozogamicin achieves high remission and minimal residual disease negativity rates, although it requires careful monitoring due to serious adverse events such as sinusoidal obstruction syndrome. The bispecific T-cell engager blinatumomab induces deep remission through T-cell-mediated cytotoxicity but is associated with cytokine release syndrome and neurotoxicity. These immunotherapies play a crucial role as a bridge to allogeneic hematopoietic stem cell transplantation, and timely transition to transplantation is essential for improving prognosis. CAR-T therapy has demonstrated high response rates and durable remissions; however, its use is limited by eligibility and access. Further optimization of frontline therapy, as well as development and appropriate integration of novel therapeutic approaches, is warranted.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 9","pages":"1132-1137"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149290603","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}

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[Chronic active Epstein-Barr virus disease: current understanding of its pathogenesis, diagnosis, and treatment]. [慢性活动性eb病毒病：目前对其发病机制、诊断和治疗的认识]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.1182

Ayako Arai

{"title":"[Chronic active Epstein-Barr virus disease: current understanding of its pathogenesis, diagnosis, and treatment].","authors":"Ayako Arai","doi":"10.11406/rinketsu.67.1182","DOIUrl":"https://doi.org/10.11406/rinketsu.67.1182","url":null,"abstract":"Chronic active Epstein-Barr virus disease (CAEBV) is a rare and intractable disorder characterized by persistent or recurrent inflammation associated with persistent EBV infection of T or NK cells and their clonal proliferation. In the revised fourth edition of the WHO classification, CAEBV was classified as an EBV-positive T- and NK-cell neoplasm, which greatly raised global awareness and recognition of this disease. Accurate diagnosis requires identification of the EBV-infected cell population. In addition to pathological evaluation and detection of EBV genomes in individual lymphocyte subsets by PCR, the introduction of EBER-flow FISH using flow cytometry is expected to provide more efficient diagnostic options. Today, allogeneic hematopoietic stem cell transplantation is the only established curative treatment. However, recent advances in the understanding of the molecular pathogenesis of CAEBV have led to the identification of novel therapeutic candidates tailored to the disease biology, such as JAK inhibitors. The pathogenesis of CAEBV is also being progressively elucidated. The absence of a CAEBV-specific viral strain suggests the involvement of host factors in disease development. In recent years, cases have been reported from various regions of the world, and further progress through international collaborative research is anticipated.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 9","pages":"1182-1193"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149290606","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}

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[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.507

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[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.183

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[Aggressive non-nodal mantle cell lymphoma with challenging subtype classification at presentation]. 侵袭性非结套细胞淋巴瘤，表现时亚型分类具有挑战性。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.525

Akari Yoda, Ayato Sumioka, Ibuki Takatsuka, Yujiro Ito, Kazuyuki Shigeno, Masamitsu Takaba, Shinya Fujisawa, Kensuke Naito

{"title":"[Aggressive non-nodal mantle cell lymphoma with challenging subtype classification at presentation].","authors":"Akari Yoda, Ayato Sumioka, Ibuki Takatsuka, Yujiro Ito, Kazuyuki Shigeno, Masamitsu Takaba, Shinya Fujisawa, Kensuke Naito","doi":"10.11406/rinketsu.67.525","DOIUrl":"https://doi.org/10.11406/rinketsu.67.525","url":null,"abstract":"A 57-year-old man presented with neutropenia, circulating blast-like cells, anemia, and fever. Bone marrow examination revealed that 62.8% of nucleated cells were small lymphoid blast-like cells, whose surface markers and immunophenotype were consistent with mantle cell lymphoma (MCL). Because no nodal involvement was detected at the initial visit, leukemic non-nodal MCL (nnMCL) was initially considered. However, 18F-fluorodeoxyglucose positron emission tomography demonstrated diffuse bone uptake and new nodal lesions that had not been apparent at presentation. The presence of SOX11 positivity, a high Ki-67 index, a complex karyotype, and the rapidly progressive clinical course were atypical for nnMCL and instead resembled conventional MCL. Although differentiation was challenging, a treatment protocol for aggressive conventional MCL was followed. The patient received bendamustine and rituximab, followed by CHASE-R (cyclophosphamide, cytarabine, dexamethasone, etoposide, and rituximab), and then high-dose chemotherapy with autologous peripheral blood stem cell transplantation. This case was considered borderline between conventional MCL and nnMCL. Although a definitive classification was not made, the treatment strategy was determined based on the pathological features, chromosomal abnormalities, and clinical behavior, following the approach typically used for aggressive conventional MCL.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 6","pages":"525-530"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414311","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}

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[Clinical significance of comprehensive genomic profiling in hematopoietic failure]. [综合基因组谱分析在造血衰竭中的临床意义]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.574

Satoru Aoyama

{"title":"[Clinical significance of comprehensive genomic profiling in hematopoietic failure].","authors":"Satoru Aoyama","doi":"10.11406/rinketsu.67.574","DOIUrl":"https://doi.org/10.11406/rinketsu.67.574","url":null,"abstract":"This review outlines the clinical significance of comprehensive genomic profiling (CGP) in patients with hematopoietic failure, including severe unexplained cytopenia. Since March 2025, Japanese National Health Insurance has covered CGP for hematologic malignancies and related disorders, facilitating a genome-based precision medicine approach that complements conventional clinical approaches, including morphologic and cytogenetic analyses. In bone marrow failure phenotypes, CGP supports differentiation of aplastic anemia from myelodysplastic syndromes and related diseases by evaluating genetic alterations. In addition to diagnosis, CGP contributes to prognostic stratification and evaluation of inherited bone marrow failure syndromes, which may present with minimal extra-hematologic features. However, accurate interpretation of CGP results requires not only understanding the genetic pathology of each disease, but also awareness of the characteristics of the CGP and the pitfalls specific to hematopoietic diseases (such as clonal hematopoiesis) and discussions with an expert panel. This review aims to facilitate the interpretation of CGP results to optimize clinical management for patients with hematopoietic failure.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 6","pages":"574-581"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148414323","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}

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[Recent advances in pathogenesis, diagnosis, and therapeutic strategies for POEMS syndrome]. 【POEMS综合征的发病机制、诊断和治疗策略的最新进展】。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.453

Shokichi Tsukamoto

{"title":"[Recent advances in pathogenesis, diagnosis, and therapeutic strategies for POEMS syndrome].","authors":"Shokichi Tsukamoto","doi":"10.11406/rinketsu.67.453","DOIUrl":"10.11406/rinketsu.67.453","url":null,"abstract":"POEMS syndrome is a rare multisystemic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell dyscrasia, and skin changes. Markedly elevated serum vascular endothelial growth factor (VEGF) levels play a crucial role in its pathogenesis. Recent genomic studies have identified specific mutational patterns in the immunoglobulin lambda light chain gene (e.g., IGLV1-40 or IGLV1-44), providing new insights into the unique biology of POEMS syndrome. For clinical diagnosis, the Japanese diagnostic criteria are highly practical and accurate, designating polyneuropathy, monoclonal plasma cell proliferation, and elevated serum VEGF levels (>1,000 pg/ml) as mandatory major criteria. Treatment strategies are determined based on eligibility for autologous stem cell transplantation (ASCT). For eligible patients, high-dose melphalan followed by ASCT is the standard of care, yielding significant neurological improvement and favorable long-term survival. In contrast, for transplant-ineligible patients or as induction therapy before ASCT, novel agents such as thalidomide, lenalidomide, and bortezomib have demonstrated clinical efficacy. Serum VEGF levels serve as an essential biomarker that sensitively reflects disease activity and treatment response, making them indispensable for monitoring during follow-up. This review provides a comprehensive overview of recent advances in the pathogenesis, diagnostic refinements, and latest therapeutic strategies for POEMS syndrome based on recent scientific evidence.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 5","pages":"453-461"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148159419","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}

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[Successful treatment of cardiac tamponade due to/pericardial infiltration with pericardial drainage and intrapericardial chemotherapy in a patient with acute myeloid leukemia]. 【心包引流加心包内化疗治疗急性髓性白血病心包浸润性心包填塞一例成功】。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.97

Hiroaki Zushi, Hiroyuki Kuroda, Kosuke Nishibori, Kana Nagashima, Yuji Kanisawa, Shouya Ino, Kazuyuki Murase, Koichi Takada, Masayoshi Kobune

{"title":"[Successful treatment of cardiac tamponade due to/pericardial infiltration with pericardial drainage and intrapericardial chemotherapy in a patient with acute myeloid leukemia].","authors":"Hiroaki Zushi, Hiroyuki Kuroda, Kosuke Nishibori, Kana Nagashima, Yuji Kanisawa, Shouya Ino, Kazuyuki Murase, Koichi Takada, Masayoshi Kobune","doi":"10.11406/rinketsu.67.97","DOIUrl":"10.11406/rinketsu.67.97","url":null,"abstract":"A 66-year-old man with acute myeloid leukemia with myelodysplasia-related changes (AML-MR) was admitted in April 2024 after presenting with increased peripheral blood blasts. At the initial visit, he had atrial fibrillation but no pericardial effusion. Although treatment with venetoclax and azacitidine reduced peripheral blood blasts, a concurrent cardiac tamponade was observed in May of the same year. Pericardial drainage was performed, and cytology and flow cytometry of the pericardial fluid confirmed a diagnosis of pericardial infiltration due to AML. Pericardial effusion resolved with repeated intrapericardial injections of cytarabine, allowing discontinuation of pericardial drainage. Remission induction therapy with daunorubicin and cytarabine was subsequently administered. Palliative care for AML was given during the cardiac treatment, and pericardial effusion did not occur prior to the patient's death in February 2025.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 2","pages":"97-103"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147358031","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}

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[Key points in selecting first-line therapy for chronic myeloid leukemia]. 慢性髓系白血病一线治疗选择要点

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2026-01-01 DOI: 10.11406/rinketsu.67.130

Shinya Kimura

{"title":"[Key points in selecting first-line therapy for chronic myeloid leukemia].","authors":"Shinya Kimura","doi":"10.11406/rinketsu.67.130","DOIUrl":"10.11406/rinketsu.67.130","url":null,"abstract":"Chronic myeloid leukemia (CML) is driven by the BCR::ABL1 fusion gene, and the introduction of tyrosine kinase inhibitors (TKIs) has dramatically improved long-term survival. In Japan, five agents-imatinib (Glivec®), dasatinib (Sprycel®), nilotinib (Tasigna®), bosutinib (Bosulif®), and STAMP inhibitor asciminib (Scemblix®)-are currently approved for first-line therapy. Each has distinct efficacy and toxicity profiles, requiring individualized treatment decisions based on age, comorbidities, cardiovascular risk, fertility, adherence, and financial factors. Recent advances have shifted treatment goals from disease control to treatment-free remission (TFR), with imatinib, dasatinib, and nilotinib supported by robust clinical trial data. Emerging strategies, such as low-dose regimens and step-up dosing of TKI, highlight the importance of balancing efficacy with tolerability and quality of life. Looking forward, immunologic determinants of TFR and novel therapeutic approaches, including targeting CML stem cells with agents such as asciminib or demethylating drugs, may offer prospects for cure. This review summarizes the current evidence and practical considerations in selecting first-line therapy for chronic-phase CML, with a focus on optimizing long-term outcomes and advancing toward individualized and potentially curative strategies.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"67 2","pages":"130-141"},"PeriodicalIF":0.0,"publicationDate":"2026-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147358033","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