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

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[Cord blood transplantation: current status and future perspectives]. [脐带血移植:现状与未来展望]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.331
Takaaki Konuma
{"title":"[Cord blood transplantation: current status and future perspectives].","authors":"Takaaki Konuma","doi":"10.11406/rinketsu.66.331","DOIUrl":"10.11406/rinketsu.66.331","url":null,"abstract":"<p><p>Cord blood, which is rich in hematopoietic stem/progenitor cells, is now an important cell source for allogeneic hematopoietic cell transplantation (HCT). Although cord blood transplantation (CBT) was initially only performed in pediatric patients due to the limited number of cells, it now accounts for around 30% of allogeneic HCT for adults in Japan. It has overcome the shortage of donors caused by the declining birthrate and aging population. Although the greatest disadvantage of CBT is the high incidence of Engraftment failure, it has been shown that even in HLA-incompatible recipients, graft-versus-host disease (GVHD) is less frequent and less severe in CBT, whereas the graft-versus-leukemia (GVL) effect is stronger. CBT in Japan differs from that in other countries in that a single-unit is used in adult patients, and CBT is actively performed in elderly patients and patients with advanced hematopoietic disease. Based on the Japanese analysis, the usefulness of intensified conditioning regimen, the survival-improving effect on mild acute or chronic GVHD, and the strong GVL effect are all characteristic of CBT, which is an important source of cells for allogeneic HCT.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 5","pages":"331-338"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144227924","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
[Novel second-line treatments for immune thrombocytopenia]. [新的二线治疗免疫性血小板减少症]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.473
Hisashi Kato
{"title":"[Novel second-line treatments for immune thrombocytopenia].","authors":"Hisashi Kato","doi":"10.11406/rinketsu.66.473","DOIUrl":"10.11406/rinketsu.66.473","url":null,"abstract":"<p><p>Immune thrombocytopenia (ITP) is a thrombocytopenic disorder in which anti-platelet antibodies accelerate platelet destruction and impair platelet production. Treatment consists of first-line steroids, followed by second-line therapy if the patient is unresponsive or intolerant to steroids. Thrombopoietin receptor agonists, rituximab, and splenectomy have been the main second-line options. However, the Syk inhibitor fostamatinib and the FcRn inhibitor efgartigimod, two novel agents that are expected to improve platelet counts through a new mechanism of action, have recently been added as second-line treatment options in Japan as well. Many new therapeutic agents for ITP such as BTK inhibitors, BAFF receptor inhibitors, and anti-CD38 antibody agents are also under development. It is hoped that these novel treatment options with different effects will improve the management of ITP in the future.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"473-480"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577211","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
[Plasma exchange-free therapeutic management of immune-mediated thrombotic thrombocytopenic purpura]. [免疫介导的血栓性血小板减少性紫癜的血浆交换治疗管理]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2025-01-01 DOI: 10.11406/rinketsu.66.481
Kazuya Sakai
{"title":"[Plasma exchange-free therapeutic management of immune-mediated thrombotic thrombocytopenic purpura].","authors":"Kazuya Sakai","doi":"10.11406/rinketsu.66.481","DOIUrl":"https://doi.org/10.11406/rinketsu.66.481","url":null,"abstract":"<p><p>Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic disorder. Immune-mediated TTP is caused by autoantibodies against ADAMTS13, an enzyme responsible for cleaving von Willebrand factor (VWF). Therapeutic plasma exchange (TPE) combined with corticosteroids has historically been the standard treatment, and has significantly improved patient survival. Caplacizumab, a nanobody targeting the VWF A1 domain, inhibits platelet-VWF interactions. Addition of caplacizumab to standard therapy has led to rapid platelet count recovery and prevention of thrombotic events in acute phases. Caplacizumab combined with immunosuppressive therapy has been effective in patients unable to undergo TPE due to clinical or logistical challenges, such as anaphylaxis to fresh frozen plasma or religious restrictions. A retrospective study in 2024 reported that 90.5% of patients achieved clinical response without TPE, highlighting the potential for plasma exchange-free management of acute immune-mediated TTP. These findings underscore the growing importance of caplacizumab in modern TTP therapy. Currently, a phase 3 clinical trial (MAYARI) is evaluating the efficacy of caplacizumab and immunosuppressive therapy without TPE in treating acute immune-mediated TTP. This trial aims to validate a simplified treatment paradigm, potentially transforming the management of TTP by offering safe and effective alternatives to TPE.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"66 6","pages":"481-487"},"PeriodicalIF":0.0,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144577216","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
[Multiple myeloma with t (8;14) and t (11;14) and extramedullary infiltration of multiple organs]. [多发性骨髓瘤,伴有 t (8;14) 和 t (11;14) 以及多个器官的髓外浸润]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.492
Shinnosuke Tokuda, Koshiro Sakamoto, Makiko Mizuguchi, Yasunobu Okamoto, Hikaru Yagi, Kumiko Kagawa, Hironobu Shibata, Hideko Endo, Shuji Ozaki
{"title":"[Multiple myeloma with t (8;14) and t (11;14) and extramedullary infiltration of multiple organs].","authors":"Shinnosuke Tokuda, Koshiro Sakamoto, Makiko Mizuguchi, Yasunobu Okamoto, Hikaru Yagi, Kumiko Kagawa, Hironobu Shibata, Hideko Endo, Shuji Ozaki","doi":"10.11406/rinketsu.65.492","DOIUrl":"10.11406/rinketsu.65.492","url":null,"abstract":"<p><p>A 69-year-old man presented with lumbago and was diagnosed with multiple myeloma (IgD-λ type, R-ISS stage II) with bone-destructive lesions in the lumbar spine and sacrum. Chromosome analysis showed t (8;14)(q24;q32) and t (11;14)(q13;q32). Treatment with daratumumab, lenalidomide, and dexamethasone resulted in partial response, but the disease relapsed, with a copy number increase in t (11;14) and abnormal amplification of the 1q21 region. The patient was treated for CMV enteritis, and was admitted to the hospital due to sudden abdominal pain. Gastrointestinal perforation was diagnosed by CT scan showing free air and wall thickening in the small intestine. Emergency surgery was performed, and the tumors in the perforated area were positive for CCND1 but negative for MYC on immunostaining. The patient's general condition did not improve after the surgery and he died. Pathological autopsy revealed extramedullary infiltration of multiple organs in addition to the small intestine. Extramedullary infiltration is thought to be caused by clonal evolution, and further research is warranted to clarify its pathogenesis and establish effective therapeutic strategies in high-risk patients.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 6","pages":"492-497"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499874","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
[Successful remission induction with reduced-dose all-trans retinoic acid for acute promyelocytic leukemia complicated by COVID-19]. [用减量全反式维甲酸成功诱导并发 COVID-19 的急性早幼粒细胞白血病患者缓解病情]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.498
Rintaro Fujimoto, Tsuyoshi Kamae, Kimimori Kamijo, Masato Yasumi, Takahiro Karasuno
{"title":"[Successful remission induction with reduced-dose all-trans retinoic acid for acute promyelocytic leukemia complicated by COVID-19].","authors":"Rintaro Fujimoto, Tsuyoshi Kamae, Kimimori Kamijo, Masato Yasumi, Takahiro Karasuno","doi":"10.11406/rinketsu.65.498","DOIUrl":"10.11406/rinketsu.65.498","url":null,"abstract":"<p><p>A 43-year-old man with pancytopenia was diagnosed with acute promyelocytic leukemia (APL). On the first day of induction therapy with all-trans retinoic acid (ATRA) alone, he presented with high fever and was found to have coronavirus disease 2019 (COVID-19) infection by SARS-CoV2 antigen test. While it is generally recommended to delay treatment for APL patients with COVID-19 unless urgent APL treatment is required, this patient needed to continue treatment due to APL-induced disseminated intravascular coagulation (DIC). Considering the challenge of distinguishing between differentiation syndrome (DS) and COVID-19 exacerbation, the ATRA dosage was reduced to 50%. The patient was able to continue treatment without development of DS or exacerbation of DIC, leading to his recovery from COVID-19 and remission of APL.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 6","pages":"498-501"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499880","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
[Prognostic impact of early initiation of chemotherapy after brain biopsy in primary central nervous system lymphoma]. [原发性中枢神经系统淋巴瘤脑活检后尽早开始化疗的预后影响]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1247
Yasutaka Masuda, Ken Morita, Mineo Kurokawa
{"title":"[Prognostic impact of early initiation of chemotherapy after brain biopsy in primary central nervous system lymphoma].","authors":"Yasutaka Masuda, Ken Morita, Mineo Kurokawa","doi":"10.11406/rinketsu.65.1247","DOIUrl":"https://doi.org/10.11406/rinketsu.65.1247","url":null,"abstract":"<p><p>Primary central nervous system lymphoma (PCNSL) is a B-cell lymphoma confined to the CNS. In general, early initiation of treatment after diagnosis is considered to be associated with improved prognosis in patients with malignancy. However, the prognostic impact of the time from diagnostic brain biopsy to initiation of treatment in patients with PCNSL remains unclear. In this study, we retrospectively analyzed how time from diagnostic biopsy to rituximab-containing treatment correlated with prognosis in 19 patients with PCNSL. Patients with a better prognostic score defined by age and performance status at diagnosis tended to have a better prognosis if chemotherapy was initiated sooner after brain biopsy. In conclusion, earlier chemotherapy initiation after brain biopsy and definitive diagnosis should be considered in the treatment of PCNSL.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 10","pages":"1247-1252"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142592217","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
[Diagnosis and treatment of aplastic anemia and lower-risk myelodysplastic neoplasms]. [再生障碍性贫血和低风险骨髓增生异常肿瘤的诊断和治疗]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.884
Takahiro Suzuki
{"title":"[Diagnosis and treatment of aplastic anemia and lower-risk myelodysplastic neoplasms].","authors":"Takahiro Suzuki","doi":"10.11406/rinketsu.65.884","DOIUrl":"https://doi.org/10.11406/rinketsu.65.884","url":null,"abstract":"<p><p>Myelodysplastic neoplasms (MDS) are clonal hematological malignancies arising from gene mutations. Immunosuppressive therapies (IST) are effective in lower-risk MDS (LR-MDS) with characteristics such as hypoplastic marrow with low blasts or low ring sideroblasts, and with a small increase of PNH clones or decrease of megakaryocytes. Differential diagnosis of these LR-MDS cases from AA can be difficult, and precise diagnosis requires careful evaluation of bone marrow cellularity and dysplasia. To decide on an appropriate treatment strategy for LR-MDS, it is important to evaluate the underlying pathology, and preferentially select IST as first-line therapy in patients with features that indicate immune-mediated bone marrow failure.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 9","pages":"884-891"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142367807","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
[Fertility preservation in patients with hematopoietic diseases]. [造血疾病患者的生育能力保护]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1025
Yuma Tada
{"title":"[Fertility preservation in patients with hematopoietic diseases].","authors":"Yuma Tada","doi":"10.11406/rinketsu.65.1025","DOIUrl":"https://doi.org/10.11406/rinketsu.65.1025","url":null,"abstract":"<p><p>The prognosis of hematopoietic diseases has been improving over the past several decades, and measures to fulfill patients' wishes to have children are needed to improve survivors' quality of life. In Japan, the Japan Society for Fertility Preservation has taken the lead in developing guidelines, a national registry, and a nationwide uniform subsidy system. When considering fertility preservation in patients with hematopoietic diseases, interdisciplinary collaboration between oncology and onco-fertility is necessary to solve problems specific to hematopoietic diseases, such as the short time window between the onset of disease and the start of treatment, complications associated with fertility preservation treatment such as bleeding or ovarian hyperstimulation syndrome, and contamination of collected tissue with hematopoietic tumor cells. This article outlines the basic concept of fertility preservation in patients with hematopoietic diseases and recently established strategies for women with chronic myelogenous leukemia. It will also share some treatment innovations that can be considered in cases when fertility preservation treatment may be challenging.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 9","pages":"1025-1032"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142367809","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
[Management of children and adolescents receiving CAR-T cell therapy for acute lymphoblastic leukemia]. [接受 CAR-T 细胞疗法治疗急性淋巴细胞白血病的儿童和青少年的管理]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1164
Chihaya Imai
{"title":"[Management of children and adolescents receiving CAR-T cell therapy for acute lymphoblastic leukemia].","authors":"Chihaya Imai","doi":"10.11406/rinketsu.65.1164","DOIUrl":"https://doi.org/10.11406/rinketsu.65.1164","url":null,"abstract":"<p><p>Tisagenlecleucel, a commercially available CD19-targeted CAR-T cell product, has dramatically changed the treatment of relapsed/refractory B-cell acute lymphoblastic leukemia (B-ALL). Tisagenlecleucel infusion has been linked to distinct acute adverse events, including cytokine release syndrome, neurotoxicity, hemophagocytic lymphohistiocytosis and prolonged pancytopenia, which are rare with cytocidal chemotherapy. In addition, recent retrospective studies have revealed pre-infusion prognostic factors including high tumor burden (bone marrow leukemia cell fraction ≥5%) and non-response to blinatumomab, another CD19-targeting agent. Not only physicians providing CAR-T cell therapy but also those referring patients for this therapy should thoroughly understand the indications and limitations, characteristic acute complications, pre-treatment factors affecting prognosis, and late complications. This article outlines the current understanding regarding the use of tisagenlecleucel in children and adolescents with B-ALL.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 9","pages":"1164-1173"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142367814","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
[Basics of FCM analysis for leukemia diagnosis]. [用于白血病诊断的 FCM 分析基础]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1227
Takao Deguchi
{"title":"[Basics of FCM analysis for leukemia diagnosis].","authors":"Takao Deguchi","doi":"10.11406/rinketsu.65.1227","DOIUrl":"10.11406/rinketsu.65.1227","url":null,"abstract":"<p><p>Flow cytometry (FCM) remains an essential test in the diagnosis of leukemia despite advances in genomic testing. However, the role of FCM results as a risk factor is already extremely limited. International diagnostic criteria for leukemia already prioritize diagnosis based on genetic abnormalities, with FCM diagnosis only serving as an aid to morphological diagnosis for subtypes without genetic abnormalities. However, rapid lineage diagnosis of leukemia by FCM remains important for selecting initial treatment. FCM is also an important tool for evaluating response to molecular targeted therapy, which requires repeated measurements and rapid results. Furthermore, FCM enables prediction of specific genetic abnormalities by immunophenotypic patterns, which could make it useful for verifying the clinical impact of genetic abnormalities detected by multi-gene panel testing.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 9","pages":"1227-1233"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142367794","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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