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

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[Plasmablastic lymphoma presenting with plasmacytosis and polyclonal hypergammopathy]. [浆细胞性淋巴瘤伴有浆细胞增多症和多克隆性高炎症]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.95
Keiichi Uraisami, Masuho Saburi, Katsuya Kawano, Yosuke Kodama, Hiroyuki Takata, Yasuhiko Miyazaki, Junpei Wada, Shogo Urabe, Eiichi Ohtsuka
{"title":"[Plasmablastic lymphoma presenting with plasmacytosis and polyclonal hypergammopathy].","authors":"Keiichi Uraisami, Masuho Saburi, Katsuya Kawano, Yosuke Kodama, Hiroyuki Takata, Yasuhiko Miyazaki, Junpei Wada, Shogo Urabe, Eiichi Ohtsuka","doi":"10.11406/rinketsu.65.95","DOIUrl":"10.11406/rinketsu.65.95","url":null,"abstract":"<p><p>A 72-year-old woman presented with generalized lymphadenopathies and plasmacytosis accompanied by polyclonal hypergammopathy. <sup>18</sup>F-fluorodeoxyglucose positron emission tomography (FDG-PET) showed FDG accumulation in the systemic lymph nodes, spleen, and multiple bones. Human immunodeficiency virus antibody was negative. Lymph node histologic findings showed a monotonous population of plasma cells with a starry-sky appearance. The cells were positive for CD19, λ, and Epstein-Barr virus-encoded RNA, and negative for CD20 and CD56. The MIB-1 index was 80%. A diagnosis of plasmablastic lymphoma with plasmacytosis and polyclonal hypergammopathy was made, and complete metabolic response was achieved after six cycles of dose-adjusted-EPOCH therapy (etoposide, prednisolone, vincristine, cyclophosphamide, and doxorubicin).</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140051217","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 : 2024-01-01 DOI: 10.11406/rinketsu.65.102
{"title":"","authors":"","doi":"10.11406/rinketsu.65.102","DOIUrl":"10.11406/rinketsu.65.102","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140051265","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 : 2024-01-01 DOI: 10.11406/rinketsu.65.603
{"title":"","authors":"","doi":"10.11406/rinketsu.65.603","DOIUrl":"https://doi.org/10.11406/rinketsu.65.603","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499863","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
[Current state of graft-versus-host disease prophylaxis with PTCy for allogeneic hematopoietic stem cell transplantation]. [异基因造血干细胞移植中使用 PTCy 预防移植物抗宿主病的现状]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.391
Hirohisa Nakamae
{"title":"[Current state of graft-versus-host disease prophylaxis with PTCy for allogeneic hematopoietic stem cell transplantation].","authors":"Hirohisa Nakamae","doi":"10.11406/rinketsu.65.391","DOIUrl":"10.11406/rinketsu.65.391","url":null,"abstract":"<p><p>There is growing recognition of post-transplant cyclophosphamide (PTCy) as the new standard prophylaxis for graft-versus-host disease (GVHD) in HLA-matched peripheral blood stem cell transplants with reduced intensity conditioning, based on recent results of randomized phase III trials of PTCy. Allogeneic hematopoietic cell transplantation (HCT) with PTCy is thought to have GVHD-dependent and -independent graft-versus-tumor (GVT) effects. Its GVHD-dependent effects may be attenuated by PTCy-induced alloreactive T cell dysfunction and preferential recovery of regulatory T cells after HCT, but its GVT effects do not appear to be significantly impaired in patients in remission or with indolent disease. As patients not in remission are often also candidates for transplantation in Japan, it will be necessary to use PTCy as a platform to establish a strategy that could also be effective in patients not in remission and to revise the donor selection algorithm.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141201455","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
[Cold agglutinin disease: pathology, diagnosis, and treatment]. [冷凝集素病:病理学、诊断和治疗]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.521
Hideho Wada
{"title":"[Cold agglutinin disease: pathology, diagnosis, and treatment].","authors":"Hideho Wada","doi":"10.11406/rinketsu.65.521","DOIUrl":"10.11406/rinketsu.65.521","url":null,"abstract":"<p><p>Cold agglutinin disease (CAD), an immune hemolytic disease mediated by the classical complement-dependent pathway, accounts for approximately 8% of autoimmune hemolytic anemia (AIHA) cases. Primary CAD is a clonal B-cell lymphoproliferative disease of the bone marrow that produces IgM type-M protein, while conventional secondary CAD is cold agglutinin syndrome (CAS). Clinical findings are broadly classified into chronic anemia due to hemolysis and symptoms associated with peripheral circulatory failure due to erythrocyte aggregation under cold exposure. Not all patients require drug therapy, but monoclonal antibody therapy against complement C1s is preferred for the former presentation and B-cell suppressors for the latter. As cold AIHA is treated differently than warm AIHA, misdiagnosis can significantly impact the outcome of treatment. The most important aspect of blood testing is temperature control of specimens. Cold agglutinin titer, IgM quantification, electrophoresis, and immunofixation methods may produce false-negative results if the serum is not temperature-controlled at 37-38°C until serum separation. Correct handling of specimens, along with knowledge of the various clinical features of CAD, will lead to correct diagnosis and appropriate treatment.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499870","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
[Durable remission with lenalidomide in a patient with early relapse of adult T-cell leukemia/lymphoma after cord blood transplantation]. [脐带血移植后成人T细胞白血病/淋巴瘤早期复发患者使用来那度胺可获得持久缓解]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.628
Masakazu Mori, Yuki Goto, Ryuichiro Hiyama, Ryo Ueda, Risa Hashida, Kyoko Itakusu, Kyosuke Saeki, Koichi Nakase, Yuichiro Nawa
{"title":"[Durable remission with lenalidomide in a patient with early relapse of adult T-cell leukemia/lymphoma after cord blood transplantation].","authors":"Masakazu Mori, Yuki Goto, Ryuichiro Hiyama, Ryo Ueda, Risa Hashida, Kyoko Itakusu, Kyosuke Saeki, Koichi Nakase, Yuichiro Nawa","doi":"10.11406/rinketsu.65.628","DOIUrl":"10.11406/rinketsu.65.628","url":null,"abstract":"<p><p>A 62-year-old woman with adult T-cell leukemia/lymphoma (ATL) received umbilical cord blood transplantation (CBT) in first complete remission. However, relapse of ATL was detected on day 74 post-transplantation, as evidenced by the rapid growth of lymphoma cells in peripheral blood and an increase in soluble interleukin-2 receptor (sIL2R) levels. Discontinuation of immunosuppressant therapy alone did not improve ATL findings, but treatment with lenalidomide caused lymphoma cells to disappear from the peripheral blood and sIL2R levels to return to normal. Pancytopenia was observed as a lenalidomide-associated adverse effect, but lymphocyte counts were not reduced. The patient was judged to be in complete remission based on results of Southern blot analysis and human T-cell leukemia virus 1 (HTLV-1)-infected cell analysis using flow cytometry (HAS-Flow). Flow cytometric analysis of peripheral blood and FISH analysis of X and Y chromosomes revealed that the therapeutic effect of lenalidomide was associated with an increase in the number of donor-derived peripheral natural killer cells. ATL relapse was not observed at 13 months into lenalidomide treatment. Our results suggest that lenalidomide is an effective option for the treatment of post-transplant relapsed ATL.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141891240","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 : 2024-01-01 DOI: 10.11406/rinketsu.65.453
{"title":"","authors":"","doi":"10.11406/rinketsu.65.453","DOIUrl":"https://doi.org/10.11406/rinketsu.65.453","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141201449","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
[Congenital thrombotic thrombocytopenic purpura diagnosed in adulthood after repeated thrombocytopenia since neonatal period]. [先天性血栓性血小板减少性紫癜,自新生儿期起反复血小板减少,成年后确诊]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.142
Teruhiko Yoshino, Takuro Kuriyama, Sae Utsumi, Takashi Shimakawa, Mariko Minami, Masayasu Hayashi, Yayoi Matsuo, Koichi Kokame, Eriko Nakamura, Masanori Matsumoto, Tetsuya Eto, Shuichi Taniguchi
{"title":"[Congenital thrombotic thrombocytopenic purpura diagnosed in adulthood after repeated thrombocytopenia since neonatal period].","authors":"Teruhiko Yoshino, Takuro Kuriyama, Sae Utsumi, Takashi Shimakawa, Mariko Minami, Masayasu Hayashi, Yayoi Matsuo, Koichi Kokame, Eriko Nakamura, Masanori Matsumoto, Tetsuya Eto, Shuichi Taniguchi","doi":"10.11406/rinketsu.65.142","DOIUrl":"https://doi.org/10.11406/rinketsu.65.142","url":null,"abstract":"<p><p>A 27-year-old woman was diagnosed with idiopathic thrombocytopenic purpura in the neonatal period, and was admitted to our hospital after presenting with impaired consciousness, purpura, nausea and vomiting, with a platelet count of 10×10<sup>9</sup>/l. Congenital thrombotic thrombocytopenic purpura (cTTP) was suspected on the basis of recurrent thrombocytopenia and impaired consciousness, so tests for ADAMTS13 activity and inhibitor were performed. ADAMTS13 activity was severely decreased, ADAMTS13 inhibitor was negative, and platelet count increased after transfusion of fresh frozen plasma. These findings and the results of genetic testing done on all family members led to a diagnosis of cTTP. cTTP requires differential diagnosis even in adults. If a patient diagnosed with ITP in childhood has a history or findings that suggest cTTP during follow-up observation, it is necessary to actively consider ADAMTS13 testing.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140856864","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
[Mitochondrial metabolism and erythroid differentiation]. [线粒体代谢与红细胞分化]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.183
Tohru Fujiwara
{"title":"[Mitochondrial metabolism and erythroid differentiation].","authors":"Tohru Fujiwara","doi":"10.11406/rinketsu.65.183","DOIUrl":"https://doi.org/10.11406/rinketsu.65.183","url":null,"abstract":"<p><p>The transcription factor GATA-1 is essential for erythroid differentiation. Recently, FAM210B, which encodes a mitochondrial inner membrane protein, has been identified as a novel target of GATA-1. To clarify the role of FAM210B, we depleted endogenous FAM210B in human iPS-derived erythroid progenitor (HiDEP-1) cells, and found that erythroid differentiation was more pronounced in the FAM210B depleted cells. Comprehensive metabolite analysis revealed a decline in mitochondrial function accompanied by increased lactate production, indicative of anaerobic glycolysis. Mass spectrometry revealed that FAM210B could interact with multiple subunits of mitochondrial ATP synthases, such as subunit alpha (ATP5A) and beta (ATP5B). Our results suggested that FAM210B contributes prominently to erythroid differentiation by regulating mitochondrial energy metabolism. This review will discuss the potential association between mitochondrial metabolism and erythropoiesis.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140873391","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
[Clonal evolution in myeloproliferative neoplasms]. [骨髓增生性肿瘤的克隆进化]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.784
Kenichi Yoshida
{"title":"[Clonal evolution in myeloproliferative neoplasms].","authors":"Kenichi Yoshida","doi":"10.11406/rinketsu.65.784","DOIUrl":"10.11406/rinketsu.65.784","url":null,"abstract":"<p><p>Recent advances in sequencing technologies have clarified the driver gene landscape in Philadelphia chromosomenegative (Ph-) myeloproliferative neoplasms (MPNs) and progressed understanding of MPN pathogenesis. Beyond mutations in the main three drivers of MPN, namely JAK2, MPL and CALR, somatic mutations in the epigenetic regulators and RNA splicing factors have been identified and their association with transformation to myelofibrosis and acute myeloid leukemia have been determined. Clonal expansion of hematopoietic cells with driver mutations (clonal hematopoiesis) has been detected in healthy individuals, especially in elderly people. In MPN patients, however, initial driver mutations such as those in JAK2 and DNMT3A have been shown to be acquired in utero or during childhood. In this review, I will summarize the recent findings about clonal evolution in MPN and the role of driver mutations.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142134699","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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