[Rinsho ketsueki] The Japanese journal of clinical hematology最新文献_第2页

[Successful rituximab treatment of TAFRO syndrome refractory to glucocorticoids and tocilizumab]. [利妥昔单抗成功治疗糖皮质激素和托珠单抗难治性TAFRO综合征]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.265

Chihiro Sumi, Yasumichi Toki, Takuya Funayama, Takeshi Saito, Mayumi Hatayama, Masayo Yamamoto, Motohiro Shindo, Sayaka Yuzawa, Mishie Tanino, Toshikatsu Okumura

{"title":"[Successful rituximab treatment of TAFRO syndrome refractory to glucocorticoids and tocilizumab].","authors":"Chihiro Sumi, Yasumichi Toki, Takuya Funayama, Takeshi Saito, Mayumi Hatayama, Masayo Yamamoto, Motohiro Shindo, Sayaka Yuzawa, Mishie Tanino, Toshikatsu Okumura","doi":"10.11406/rinketsu.64.265","DOIUrl":"https://doi.org/10.11406/rinketsu.64.265","url":null,"abstract":"A 53-year-old man was presented with fever, eyelid edema, and thrombocytopenia. Based on examination outcomes, he was diagnosed with immune thrombocytopenia. He was prescribed prednisolone (PSL) at 0.5 mg/kg/day; subsequently, his platelet count improved and fever improved. PSL dose was tapered and stopped without relapse. However, 1 month later, the patient presented to our hospital with fever, generalized edema, thrombocytopenia, and acute renal failure. Computed tomography revealed multiple lymphadenopathies, hepatomegaly, pleural effusion, and ascites. Bone marrow biopsy indicated reticulin fibrosis, and lymph node biopsy revealed mixed-type Castleman disease. Based on these findings, he was diagnosed with grade 5 TAFRO syndrome (very severe). Steroid pulse therapy and tocilizumab were ineffective in improving his condition. Therefore, rituximab was administered instead of tocilizumab, and his condition eventually improved. The optimal treatment for TAFRO syndrome is yet to be established. If tocilizumab is ineffective as the second-line treatment, then rituximab might be effective.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 4","pages":"265-270"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9384945","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 : 2023-01-01 DOI: 10.11406/rinketsu.64.243

引用次数: 0

[Successful treatment of traumatic intramuscular hemorrhage with coagulation factor VIII replacement in a patient with congenital hemophilia A with decreased inhibitor activity by emicizumab therapy]. [用凝血因子VIII替代半珠单抗治疗抑制剂活性降低的先天性血友病a患者创伤性肌内出血的成功治疗]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.198

Takeshi Kageyama, Makiko Mizuguchi, Yasunobu Okamoto, Hikaru Yagi, Kumiko Kagawa, Hironobu Shibata, Shuji Ozaki

引用次数: 0

[Autoimmune coagulation factor V/5 deficiency during chronic disseminated intravascular coagulation]. 慢性弥散性血管内凝血过程中自身免疫凝血因子V/5缺乏。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.113

Noriko Ishimori, Mutsumi Wakabayashi, Kenji Sakurai, Akira Suda, Masayoshi Souri, Tsukasa Osaki, Akitada Ichinose

{"title":"[Autoimmune coagulation factor V/5 deficiency during chronic disseminated intravascular coagulation].","authors":"Noriko Ishimori, Mutsumi Wakabayashi, Kenji Sakurai, Akira Suda, Masayoshi Souri, Tsukasa Osaki, Akitada Ichinose","doi":"10.11406/rinketsu.64.113","DOIUrl":"https://doi.org/10.11406/rinketsu.64.113","url":null,"abstract":"Aortic regurgitation, a thoracoabdominal aortic aneurysm, chronic myeloid leukemia, and chronic kidney disease were all being treated at two hospitals for an 83-year-old man. He was admitted to the Department of Orthopedics at our hospital with a lumbar compression fracture. Later, he experienced melena, for which the Department of Internal Medicine was consulted. Due to the aberrant results of PT-INR (7.1) and a PTT > 200 seconds on a coagulation test, we suspected the presence of an autoimmune coagulation factor deficiency, and prednisolone immunosuppressive therapy medication was started right away. Due to a sharp decline in FV/5 activity, the presence of FV/5 inhibitors, and the presence of anti-FV/5 autoantibodies, a final diagnosis of autoimmune coagulation factor V (FV/5) deficiency was made. After the start of immunosuppressive therapy, the FV/5 inhibitor and anti-FV/5 autoantibodies disappeared, and the FV/5 activity progressively returned to normal. Disseminated intravascular coagulation-which may have been caused by a known aortic aneurysm-worsened while tapering off prednisolone. Due to the patient's advanced age and other problems, the aneurysm was extensive and inappropriate for surgical repair. The coagulation test findings improved gradually upon initiation of warfarin therapy. Herein, the patient had autoimmune FV/5 deficiency, a rare disorder that made diagnosis and therapy difficult because of the patient's several coexisting conditions.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 2","pages":"113-118"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9220908","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

[Clinical significance of clonal hematopoiesis and disease boundaries in bone marrow failure diseases]. [骨髓衰竭疾病克隆造血及疾病界限的临床意义]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.474

Takahiro Suzuki

引用次数: 0

[Elucidation of an altered anticoagulant function due to Factor V abnormality and development of a simple screening assay for thrombophilia]. [因子V异常导致抗凝血功能改变的解释和一种简单的血栓病筛查方法的发展]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.654

Naruto Shimonishi, Keiji Nogami

{"title":"[Elucidation of an altered anticoagulant function due to Factor V abnormality and development of a simple screening assay for thrombophilia].","authors":"Naruto Shimonishi, Keiji Nogami","doi":"10.11406/rinketsu.64.654","DOIUrl":"https://doi.org/10.11406/rinketsu.64.654","url":null,"abstract":"Coagulation factor V (FV) is both procoagulant and anticoagulant functions. Congenital FV abnormality, which are caused by mutations in the FV gene, are characterized by a tendency to bleed. However, FV-R506Q (FVLeiden) is the most common FV abnormality that eliminates an activated protein C (APC) cleavage site, resulting in the occurrence of deep venous thrombosis (DVT). In Japan, the thrombotic predisposition caused by FVLeiden and FV molecular abnormalities was believed to be nonexistent. We did, however, report the first case in Japan of a young patient with FV abnormality-related thrombosis. The recurrent DVT in this case was caused by a novel mutation of FV-W1920R (FVNara), located in the C1 domain and far from the APC cleavage sites. We considered the possibility that there were cases of FV-related thrombotic predisposition that had gone undetected in Japan. We thoroughly examined FV-related anticoagulant function to understand the pathogenesis of thrombosis caused by FV abnormality. Furthermore, using recombinant thrombomodulin, we successfully developed a novel assay with clot waveform analysis for the rapid detection of FV deficiency with APC resistance. Other FV abnormality-related thrombosis has been reported in Japan in recent years, and we hope to further clarify the FV-related thrombotic predisposition in the future.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 7","pages":"654-660"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9956316","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 : 2023-01-01 DOI: 10.11406/rinketsu.64.321

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[Molecular targeted therapy for acute myeloid leukemia]. [分子靶向治疗急性髓性白血病]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.345

Yuichi Ishikawa

{"title":"[Molecular targeted therapy for acute myeloid leukemia].","authors":"Yuichi Ishikawa","doi":"10.11406/rinketsu.64.345","DOIUrl":"https://doi.org/10.11406/rinketsu.64.345","url":null,"abstract":"Acute myeloid leukemia (AML) is a heterogeneous disease, and the accumulation of various chromosomal and genetic abnormalities is considerably involved in its pathogenesis and prognosis. Recently, the disease classification based on molecular abnormalities and novel molecular-targeting therapies has been developed. In Europe and the United States, several agents have been approved for AML and incorporated into guidelines as the standard treatment depending on comorbid genetic mutations combined with conventional chemotherapy or monotherapy since 2017. The combination therapy of FLT3 inhibitor midostaurin and intensive chemotherapy has improved the prognosis of patients with FLT3-ITD-positive AML, which has been considered a poor prognosis for a long period. In addition to small-molecule compounds, various novel therapies for AML are under clinical investigation, including antibody therapies targeting CD47 and TIM-3, bispecific antibodies, and CAR-T-cell therapies. Considering the treatment strategies with diverse therapeutic modalities, the pathogenesis and clonal selection process of refractory AML, including the surrounding environment of residual leukemia cells, should be clarified. The combination of new therapies and chemotherapies is highly expected to improve the prognosis of patients with AML in the near future.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 5","pages":"345-354"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9631071","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

[New concepts in multiple myeloma by returning to cytogenetics]. [从细胞遗传学角度探讨多发性骨髓瘤的新概念]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.411

Taku Tsukamoto

{"title":"[New concepts in multiple myeloma by returning to cytogenetics].","authors":"Taku Tsukamoto","doi":"10.11406/rinketsu.64.411","DOIUrl":"https://doi.org/10.11406/rinketsu.64.411","url":null,"abstract":"Multiple myeloma (MM) is characterized by genomic instability, which causes multiple genetic and chromosomal alterations and leads to disease progression and therapeutic resistance. Overlapping mechanisms, including defective genome repair machinery such as the loss of TP53 activity, as well as chromosomal segregation error represented by the abnormality of mitotic checkpoint kinases such as BUB1, cell cycle dysregulation, and tumor environment, cause structural and numerical chromosomal abnormalities. Cytogenetic abnormalities are important prognostic factors, and they are also linked to the use of proteasome inhibitors, immunomodulatory drugs, monoclonal antibodies, and the BCL2 inhibitor venetoclax. We developed new diagnostic modalities for chromosomal analysis to improve the sensitivity and convenience of chromosomal diagnosis. The immunophenotyped-suspension-multiplex (ISM)-fluorescence in situ hybridization (FISH) can use imaging flow to examine three IGH-related chromosomal translocations at the same time. We also created a new FISH method called amplified FISH (amFISH) to detect microdeletion involving narrow chromosomal regions (approximately<100 kb), such as the microdeletions of 1p32 (CDKN2C) or of 14q32 (TRAF3), by using a fluorescent antibody to amplify the signals of small probes. Even in the era of clinical sequencing, these convenient modalities may hasten the cytogenetics-oriented therapeutic approach for MM.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 5","pages":"411-417"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9631074","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

[HLA-haploidentical stem cell transplantation]. hla -单倍体干细胞移植。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.515

Junichi Sugita

{"title":"[HLA-haploidentical stem cell transplantation].","authors":"Junichi Sugita","doi":"10.11406/rinketsu.64.515","DOIUrl":"https://doi.org/10.11406/rinketsu.64.515","url":null,"abstract":"HLA-haploidentical stem cell transplantations using posttransplant cyclophosphamide (PTCy-haplo) rapidly increased worldwide. In Japan, the number of HLA-haploidentical stem cell transplantation cases exceeded related HLA-matched transplants in 2020. Recent retrospective studies using Japanese registry data have reported comparable transplantation outcomes between PTCy-haplo and HLA-matched unrelated and cord blood transplantations. PTCy-haplo was initially developed in the bone marrow transplantation setting after non-myeloablative conditioning but has recently become widely used in peripheral blood stem cell transplantation and myeloablative conditioning. Peripheral blood stem cell transplantation increases the occurrence of graft-versus-host disease but may have more improved transplant outcomes compared with bone marrow transplantation. Other factors, such as the number of infused cluster of differentiation 34-positive cells, donor age, HLA class II mismatch, HLA-B leader, and reduced PTCy dosage, may also contribute to the outcome of PTCy-haplo transplantations. Furthermore, PTCy has been reportedly effective in related/unrelated HLA-matched transplantation and HLA-mismatched unrelated transplantations. A prospective phase II trial using PTCy in patients who underwent HLA-matched and 1-2 allele-mismatched transplantation is ongoing in Japan. Patient enrollment has already been completed, and the results will be revealed soon. Using PTCy to sufficiently reduce the occurrence of graft-versus-host disease will make performing allogeneic transplants with a higher safety level possible.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 6","pages":"515-523"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9803683","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: 1