[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献_第8页

[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":"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.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 6","pages":"521-528"},"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}

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[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":"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.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 7","pages":"628-632"},"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}

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[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.453

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[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":"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 10×109/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.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 3","pages":"142-146"},"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}

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[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

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[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1492

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[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1351

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[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1428

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[Primary hepatic diffuse large B-cell lymphoma developed in a patient with primary biliary cholangitis]. [原发性肝弥漫性大b细胞淋巴瘤发生于原发性胆管炎患者]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1480

Shinichiro Watanabe, Kosuke Nakaji, Tsunehiro Ochi, Noriko Wada, Fumiya Ogasawara, Shohei Yoshida, Kensuke Kojima

{"title":"[Primary hepatic diffuse large B-cell lymphoma developed in a patient with primary biliary cholangitis].","authors":"Shinichiro Watanabe, Kosuke Nakaji, Tsunehiro Ochi, Noriko Wada, Fumiya Ogasawara, Shohei Yoshida, Kensuke Kojima","doi":"10.11406/rinketsu.65.1480","DOIUrl":"https://doi.org/10.11406/rinketsu.65.1480","url":null,"abstract":"Primary hepatic lymphoma (PHL) is a lymphoproliferative disorder confined to the liver, with no evidence of lymphomatous involvement in other organs. Here, we report a case of diffuse large B-cell lymphoma (DLBCL)-type PHL in a patient with a long history of primary biliary cholangitis (PBC) and Sjögren's syndrome (SS). A 78-year-old woman presented with epigastralgia and was found to have a solitary liver tumor by contrast-enhanced computed tomography (CT). A CT scan performed 8 months earlier was unremarkable, with no extrahepatic lesions observed. The patient underwent laparoscopic left lateral segmentectomy, and the resected specimen showed histological features of DLBCL in the background of PBC-affected liver parenchyma. The patient received postoperative R-CHOP chemotherapy and has remained in complete remission for 4 years. PBC-associated PHL is extremely rare, and five of six reported cases of PBC-associated PHL cases showed a histological subtype of MALT lymphoma. In this case, immune dysregulation caused by PBC and secondary SS may have contributed to the development of DLBCL-type PHL.","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 12","pages":"1480-1484"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142960159","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 : 2024-01-01 DOI: 10.11406/rinketsu.65.1341

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