[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

引用次数: 0

[Manufacturing results of tisagenlecleucel for acute lymphoblastic leukemia: a survey by the CAR-T cell therapy taskforce of the Japan Society of Transfusion Medicine and Cell Therapy]. [急性淋巴细胞白血病tisagenlecuucel的制造结果:日本输血医学和细胞治疗学会CAR-T细胞治疗工作组的调查]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.331

Tomoyasu Jo, Tomoko Henzan, Daisuke Tomizawa, Satoru Yoshihara, Kaoru Kahata, Minami Yamada-Fujiwara, Yoshiki Okuyama, Norio Shiba, Keiko Fujii, Yoshihiro Umezawa, Rie Yamazaki, Wataru Takeda, Ryo Hanajiri, Kentaro Fukushima, Naoya Mimura, Junko Ikemoto, Keita Iwaki, Noboru Yonetani, Shin-Ichiro Fujiwara, Masaki Ri, Tokiko Nagamura-Inoue, Ryuji Tanosaki, Yasuyuki Arai

{"title":"[Manufacturing results of tisagenlecleucel for acute lymphoblastic leukemia: a survey by the CAR-T cell therapy taskforce of the Japan Society of Transfusion Medicine and Cell Therapy].","authors":"Tomoyasu Jo, Tomoko Henzan, Daisuke Tomizawa, Satoru Yoshihara, Kaoru Kahata, Minami Yamada-Fujiwara, Yoshiki Okuyama, Norio Shiba, Keiko Fujii, Yoshihiro Umezawa, Rie Yamazaki, Wataru Takeda, Ryo Hanajiri, Kentaro Fukushima, Naoya Mimura, Junko Ikemoto, Keita Iwaki, Noboru Yonetani, Shin-Ichiro Fujiwara, Masaki Ri, Tokiko Nagamura-Inoue, Ryuji Tanosaki, Yasuyuki Arai","doi":"10.11406/rinketsu.64.331","DOIUrl":"https://doi.org/10.11406/rinketsu.64.331","url":null,"abstract":"The frequency of the manufacturing failure of chimeric antigen receptor (CAR)-T cell therapy in clinical practice is unknown. To clarify the current state of how likely CAR-T cell production is to succeed or fail for B-cell acute lymphoblastic leukemia (B-ALL), we analyzed cases in which the production of tisagenlecleucel was performed for patients with B-ALL at 15 facilities in Japan from October 2019 to March 2022. Total 81 patients (47 males and 34 females) were analyzed. The median age at apheresis was 13 years (1-25) with a median number of prior treatments of 4 (1-9). The numbers of patients with histories of allogeneic transplantation, inotuzumab ozogamicin, or blinatumomab treatments were 51 (63.0%), 26 (32.1%), and 37 (45.7%), respectively. The median blast percentage and CD3+ cell counts in peripheral blood were 0% (0-91.5), and 611/µl (35-4,210) at apheresis, and the median number of CD3+ cells shipped was 2.2×109 (0.5-8.3). While cases with a history of heavy prior treatment before apheresis were included, no manufacturing failures were observed. Continuing to monitor the status of manufacturing failures is necessary as the number of B-ALL cases treated with CAR-T cell therapy increases.","PeriodicalId":6352,"journal":{"name":"[Rinsho ketsueki] The Japanese journal of clinical hematology","volume":"64 5","pages":"331-337"},"PeriodicalIF":0.0,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9933410","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

[Molecular international prognostic scoring system for myelodysplastic syndromes]. [骨髓增生异常综合征的分子国际预后评分系统]。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.355

Yasunobu Nagata

引用次数: 98

[Vascular biology and hemophilia]. 血管生物学和血友病。

[Rinsho ketsueki] The Japanese journal of clinical hematology Pub Date : 2023-01-01 DOI: 10.11406/rinketsu.64.661

Nobuyuki Takakura

引用次数: 0