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

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[Primary myelofibrosis with double mutation in U2AF1]. [原发性骨髓纤维化伴 U2AF1 双突变]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.30
Keiko Maeyama, Keiki Nagaharu, Kazuko Ino, Yuka Sugimoto, Isao Tawara, Keiki Kawakami
{"title":"[Primary myelofibrosis with double mutation in U2AF1].","authors":"Keiko Maeyama, Keiki Nagaharu, Kazuko Ino, Yuka Sugimoto, Isao Tawara, Keiki Kawakami","doi":"10.11406/rinketsu.65.30","DOIUrl":"10.11406/rinketsu.65.30","url":null,"abstract":"<p><p>A 47-year-old woman presented with subcutaneous hemorrhage. Blood tests revealed leukoerythroblastosis, anemia, and thrombocytopenia. Bone marrow biopsy led to a diagnosis of primary myelofibrosis (aaDIPSS, DIPSS-plus: intermediate-II risk). JAK2, CALR, and MPL mutations were not detected in peripheral blood, but targeted sequencing of bone marrow specimens revealed a double mutation (Q157R, S34F) in U2AF1. Allo-PBSCT was performed using an HLA-matched related donor, and post-transplantation bone marrow examination showed complete donor chimerism on day 55. Two years after allogeneic transplantation, the patient remains relapse-free. Although U2AF1 gene abnormality is known as a poor prognostic factor in primary myelofibrosis, this patient had a favorable long-term prognosis due to prompt transplantation therapy. This case highlights the importance of detailed gene mutation analysis in patients with triple-negative MF.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 1","pages":"30-34"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139708722","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.65.197
{"title":"","authors":"","doi":"10.11406/rinketsu.65.197","DOIUrl":"https://doi.org/10.11406/rinketsu.65.197","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 3","pages":"197"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140872623","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
[Pyogenic spondylitis after Corynebacterium striatum blood stream infection following allogeneic hematopoietic stem cell transplantation for malignant lymphoma]. [因恶性淋巴瘤接受异体造血干细胞移植后,因纹状体科里奈杆菌血流感染引发化脓性脊柱炎]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.243
Takumi Nishikawa, Masuho Saburi, Kentaro Nagamatsu, Keiichi Uraisami, Hiroyuki Takata, Yasuhiko Miyazaki, Eiichi Ohtsuka
{"title":"[Pyogenic spondylitis after Corynebacterium striatum blood stream infection following allogeneic hematopoietic stem cell transplantation for malignant lymphoma].","authors":"Takumi Nishikawa, Masuho Saburi, Kentaro Nagamatsu, Keiichi Uraisami, Hiroyuki Takata, Yasuhiko Miyazaki, Eiichi Ohtsuka","doi":"10.11406/rinketsu.65.243","DOIUrl":"https://doi.org/10.11406/rinketsu.65.243","url":null,"abstract":"<p><p>Patient 1 was a 70-year-old woman with refractory diffuse large B-cell lymphoma who received allogeneic peripheral blood stem cell transplantation from an HLA-haploidentical related donor. Upper back pain appeared on day63, and Th8-Th9 pyogenic spondylitis was diagnosed based on magnetic resonance imaging (MRI). Blood culture on day14 identified Corynebacterium striatum as the causative bacteria of blood stream infection (BSI). The pyogenic spondylitis resolved after treatment with daptomycin for 2 months. Patient 2 was a 65-year-old man with relapsed angioimmunoblastic T-cell lymphoma who received bone marrow transplantation from an HLA-DR single-antigen-mismatched unrelated donor. Lower back pain appeared on day30, and L4-L5 pyogenic spondylitis was diagnosed based on MRI. Blood culture was negative. Daptomycin and clindamycin were selected for treatment based on the drug susceptibility of bacteria that had caused pre-engraftment BSI (Escherichia coli on day3 and Corynebacterium striatum on day9), and the pyogenic spondylitis resolved after 6 months of this treatment. Pyogenic spondylitis should be considered in the differential diagnosis of back pain accompanied by BSI before engraftment in allogeneic hematopoietic stem cell transplant recipients.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 4","pages":"243-248"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140873188","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
[Infection stress and a driver mutation interact to promote transformation to hematological malignancies]. [感染压力和驱动突变相互作用,促进向血液恶性肿瘤的转化]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.702
Goro Sashida
{"title":"[Infection stress and a driver mutation interact to promote transformation to hematological malignancies].","authors":"Goro Sashida","doi":"10.11406/rinketsu.65.702","DOIUrl":"https://doi.org/10.11406/rinketsu.65.702","url":null,"abstract":"<p><p>Myelodysplastic syndrome (MDS) is a refractory cancer that arises from hematopoietic stem cells and predominantly affects elderly adults. In addition to driver gene mutations, which are also found in clonal hematopoiesis in healthy elderly people, systemic inflammation caused by infection or collagen disease has long been known as an extracellular factor in the pathogenesis of MDS. Wild-type HSCs have an \"innate immune memory\" that functions in response to infection and inflammatory stress, and my colleagues and I used an infection stress model to demonstrate that the innate immune response by the TLR-TRIF-PLK-ELF1 pathway is similarly critical in impairment of hematopoiesis and dysregulation of chromatin in MDS stem cells. This revealed that not only are MDS stem cells expanded by the TRAF6-NF-kB pathway, the innate immune response is also involved in generating MDS stem cells. In this review, I will present research findings related to \"innate immune memory,\" one of the pathogenic mechanisms of blood cancer, and discuss future directions for basic pathological research and potential therapeutic development.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 7","pages":"702-708"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141891241","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
[Recent findings and advances in treatment of myeloproliferative neoplasms]. [骨髓增生性肿瘤治疗的最新发现和进展]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.375
Yuka Sugimoto
{"title":"[Recent findings and advances in treatment of myeloproliferative neoplasms].","authors":"Yuka Sugimoto","doi":"10.11406/rinketsu.65.375","DOIUrl":"10.11406/rinketsu.65.375","url":null,"abstract":"<p><p>Many novel agents have been developed for BCR::ABL1-negaive myeloproliferative neoplasms (MPN), namely, polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). Some of these agents not only achieve hematologic complete response, reduce spleen size, and alleviate constitutional symptoms, but also induce molecular response, which means that they reduce the allele burden of driver gene mutations. These agents also prevent and alleviate fibrosis in bone marrow, which reduces the incidence of thrombotic events and disease progression and might improve prognosis. This article discusses the latest findings and promising treatments, including ongoing clinical trials, in PV, ET, and PMF.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 5","pages":"375-384"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141201508","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.65.439
{"title":"","authors":"","doi":"10.11406/rinketsu.65.439","DOIUrl":"https://doi.org/10.11406/rinketsu.65.439","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 5","pages":"439-452"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141201480","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
[Overview]. [概述]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.341
Takahiro Yamauchi
{"title":"[Overview].","authors":"Takahiro Yamauchi","doi":"10.11406/rinketsu.65.341","DOIUrl":"https://doi.org/10.11406/rinketsu.65.341","url":null,"abstract":"","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 5","pages":"341-342"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141201552","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
[Advances in the treatment of thrombotic thrombocytopenic purpura]. [治疗血栓性血小板减少性紫癜的进展]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.567
Masahito Uchihara, Masayuki Kubo, Masanori Matsumoto
{"title":"[Advances in the treatment of thrombotic thrombocytopenic purpura].","authors":"Masahito Uchihara, Masayuki Kubo, Masanori Matsumoto","doi":"10.11406/rinketsu.65.567","DOIUrl":"https://doi.org/10.11406/rinketsu.65.567","url":null,"abstract":"<p><p>Thrombotic thrombocytopenic purpura (TTP) is a fatal thrombotic disease caused by a marked decrease in the activity of ADAMTS13, a von Willebrand factor cleaving protease. In congenital TTP, ADAMTS13 activity is decreased by an abnormality in ADAMTS13, and in acquired TTP, by anti-ADAMTS13 autoantibody. Death from thrombosis in the acute phase has been an issue with conventional treatment of acquired TTP by plasma exchange or immunosuppressive therapy. However, the advent of caplacizumab, an anti-VWF nanobody, has made it possible to suppress thrombus formation and is expected to improve survival rates. In addition, some case series have shown the efficacy of caplacizumab without plasma exchange for acquired TTP, and this approach is being investigated in clinical trials. Fresh-frozen plasma is transfused to supply ADAMTS13 for congenital TTP, but frequent transfusions over a long period of time can lead to problems such as infection and allergic reactions. Novel therapies such as recombinant ADAMTS13 products and gene therapy are now under development, and show promise for future clinical use.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 6","pages":"567-575"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499864","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
[Fulminant Clostridioides difficile infection during treatment with FLT3 inhibitor for acute myeloid leukemia]. [在使用 FLT3 抑制剂治疗急性髓性白血病期间出现难辨梭状芽孢杆菌感染】。]
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.153
Jotaro Yamamoto, Otoya Watanabe, Takashi Sako, Shinsuke Takagi, Daisuke Kaji, Yuki Taya, Aya Nishida, Hisashi Yamamoto, Yuki Asano-Mori, Go Yamamoto, Hideki Araoka, Naoyuki Uchida
{"title":"[Fulminant Clostridioides difficile infection during treatment with FLT3 inhibitor for acute myeloid leukemia].","authors":"Jotaro Yamamoto, Otoya Watanabe, Takashi Sako, Shinsuke Takagi, Daisuke Kaji, Yuki Taya, Aya Nishida, Hisashi Yamamoto, Yuki Asano-Mori, Go Yamamoto, Hideki Araoka, Naoyuki Uchida","doi":"10.11406/rinketsu.65.153","DOIUrl":"https://doi.org/10.11406/rinketsu.65.153","url":null,"abstract":"<p><p>An 80-year-old man with FLT3-TKD mutation-positive acute myeloid leukemia (AML) relapsed during consolidation therapy with venetoclax/azacitidine and was started on gilteritinib as salvage therapy. On the day after treatment initiation, febrile neutropenia was observed, but the fever resolved promptly after initiation of antimicrobial therapy. On the fifth day after completion of antimicrobial therapy, the patient experienced fever and watery diarrhea over 10 times a day, and a diagnosis of Clostridioides difficile infection (CDI) was made based on stool examination. The patient was treated with intravenous metronidazole, but renal dysfunction, hypotension, and hypoxemia developed, and a CT scan showed pleural and intraperitoneal effusion, significant intestinal wall thickening, and intestinal dilatation. Fidaxomicin was started under general monitoring in the intensive care unit and response was achieved. The patient was discharged from the intensive care unit on the 18th day after the onset of CDI. We report this case not only due to the rarity of fulminant CDI during AML treatment, but also because it is a valuable example of effective treatment of fulminant CDI with fidaxomicin.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 3","pages":"153-157"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140867430","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
[Clostridioides difficile infection diagnosed by nucleic acid amplification test in a patient with Epstein-Barr Virus-related T-cell lymphoproliferative disorder]. [通过核酸扩增检验诊断出一名与爱泼斯坦-巴氏病毒相关的 T 细胞淋巴增生性疾病患者感染了艰难梭菌]。
[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2024-01-01 DOI: 10.11406/rinketsu.65.732
Hideshige Seki, Megumi Yasunaga, Ken Morita, Mineo Kurokawa
{"title":"[Clostridioides difficile infection diagnosed by nucleic acid amplification test in a patient with Epstein-Barr Virus-related T-cell lymphoproliferative disorder].","authors":"Hideshige Seki, Megumi Yasunaga, Ken Morita, Mineo Kurokawa","doi":"10.11406/rinketsu.65.732","DOIUrl":"https://doi.org/10.11406/rinketsu.65.732","url":null,"abstract":"<p><p>Steroid usage poses a risk of Clostridioides difficile infection (CDI), but high-dose corticosteroid treatment can lead to false-negative CD toxin test results. Moreover, CDI-induced nausea can complicate administration of oral antibiotics, which are typically the primary therapy for CDI. In the present case, a 43-year-old woman diagnosed with EBV-associated T-cell post-transplant lymphoproliferative disorder developed CDI during treatment with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). Following five cycles of CHOP, the patient presented with nausea and diarrhea. CT scans revealed swelling in the ileocecal to transverse area of the colon. While the glutamate dehydrogenase (GDH) antigen test result was positive, the CD toxin test result was negative. However, the nucleic amplification test (NAAT) result was positive, confirming the diagnosis of CDI. Oral treatment with fidaxomicin was initially impractical due to persistent nausea. Instead, treatment began with intravenous metronidazole, and was later switched to fidaxomicin pills. Symptoms improved notably within 10 days, and the patient ultimately made a complete recovery. This case underscores the significance of exploring alternative approaches to CDI management, particularly in immunosuppressed patients.</p>","PeriodicalId":93844,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"65 8","pages":"732-736"},"PeriodicalIF":0.0,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142134700","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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