[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献_第7页

[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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[Overview]. [概述]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.1276

Masatoshi Sakurai

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[Allogeneic transplantation as a therapeutic option for atypical chronic myeloid leukemia]. [异基因移植作为非典型慢性髓性白血病的一种治疗方案]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.967

Hidehiro Itonaga

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