International Journal of Hematology最新文献

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Real-world effectiveness and safety of ibrutinib in patients with chronic lymphocytic leukemia in Japan: the Orbit study. 伊布替尼在日本慢性淋巴细胞白血病患者中的实际有效性和安全性:Orbit 研究。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-02-01 Epub Date: 2024-11-26 DOI: 10.1007/s12185-024-03875-0
Tsuyoshi Muta, Yosuke Masamoto, Go Yamamoto, Shingo Kurahashi, Yoshihiro Kameoka, Shuichi Ota, Eri Matsuki, Kazutaka Ozeki, Takanori Toyama, Naoki Takahashi, Takahiro Kumode, Nobuyuki Aotsuka, Takuro Yoshimura, Hideto Tamura, Ai Omi, Kazuhiro Shibayama, Aki Watanabe, Yasushi Isobe, Kensuke Kojima, Jun Takizawa, Hirokazu Nagai, Junji Suzumiya, Sadao Aoki
{"title":"Real-world effectiveness and safety of ibrutinib in patients with chronic lymphocytic leukemia in Japan: the Orbit study.","authors":"Tsuyoshi Muta, Yosuke Masamoto, Go Yamamoto, Shingo Kurahashi, Yoshihiro Kameoka, Shuichi Ota, Eri Matsuki, Kazutaka Ozeki, Takanori Toyama, Naoki Takahashi, Takahiro Kumode, Nobuyuki Aotsuka, Takuro Yoshimura, Hideto Tamura, Ai Omi, Kazuhiro Shibayama, Aki Watanabe, Yasushi Isobe, Kensuke Kojima, Jun Takizawa, Hirokazu Nagai, Junji Suzumiya, Sadao Aoki","doi":"10.1007/s12185-024-03875-0","DOIUrl":"10.1007/s12185-024-03875-0","url":null,"abstract":"<p><p>Ibrutinib is a first-in-class Bruton's tyrosine kinase inhibitor that is approved for the treatment of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) in Japan based on randomized clinical trial data. The aim of the real-world, retrospective Orbit study was to describe long-term clinical outcomes and management in adults (aged ≥ 20 years) with CLL/SLL treated with ibrutinib, either as first-line (1L) treatment or for relapsed or refractory (RR) disease, in routine clinical practice in Japan between July 2018 and December 2020. A total of 246 patients were registered, and the safety and per-protocol sets included 237 and 234 patients, respectively. After a median follow-up of 35.7 months, the 36-month progression-free survival rate was 80.9% in the 1L CLL cohort and 67.2% in the RR CLL cohort, and the 36-month overall survival rates were 90.8% and 83.7%, respectively. Common Terminology Criteria for Adverse Events (CTCAE) Grade ≥ 3 adverse events of special interest were atrial fibrillation (2.1%), infections (herpesvirus infection, fungal infection, or Pneumocystis jiroveci pneumonia; 1.7%), bleeding (3.8%), and second primary malignancy (2.5%). These findings confirm the long-term, real-world effectiveness and safety of ibrutinib for the treatment of Japanese patients with newly diagnosed or RR CLL/SLL.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"161-173"},"PeriodicalIF":1.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11782388/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142716193","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Comparison of busulfan pharmacokinetics between four-times-daily and once-daily administration in pediatric patients: a preliminary prospective observational trial. 一项初步前瞻性观察性试验:小儿患者每日四次和每日一次给药的丁硫凡药代动力学比较。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-02-01 Epub Date: 2024-12-03 DOI: 10.1007/s12185-024-03891-0
Atsushi Yamaguchi, Shinsuke Hirabayashi, Kazuko Niki, Keisuke Kagami, Yukayo Terashita, Yuko Cho, Atsushi Manabe, Mitsuru Sugawara, Yoh Takekuma
{"title":"Comparison of busulfan pharmacokinetics between four-times-daily and once-daily administration in pediatric patients: a preliminary prospective observational trial.","authors":"Atsushi Yamaguchi, Shinsuke Hirabayashi, Kazuko Niki, Keisuke Kagami, Yukayo Terashita, Yuko Cho, Atsushi Manabe, Mitsuru Sugawara, Yoh Takekuma","doi":"10.1007/s12185-024-03891-0","DOIUrl":"10.1007/s12185-024-03891-0","url":null,"abstract":"<p><p>Therapeutic drug monitoring (TDM) of busulfan (BU) is useful for achieving the target area under the curve (AUC) because its effective plasma-concentration range is narrow. This preliminary observational study evaluated the pharmacokinetic (PK) parameters of BU administered four times daily or once daily to pediatric patients. The plasma concentrations were measured at both the test dose and first dose, and the doses on day 1 and days 2-4 were determined based on each TDM. A comparison of PK parameters between four-times-daily and once-daily administration was performed for both the test dose and first dose of BU. Of the 11 patients, five received four-times-daily BU and six received once-daily BU. The V<sub>d</sub> for once-daily administration was higher than that for four-times-daily administration for the first dose but not for the test dose. The ratio of actual AUC for the first dose to estimated AUC guided by the test dose was lower with once-daily administration than with four-times-daily administration. These results indicate that the PK parameters of BU administered once daily are challenging to predict based on the TDM of the test dose. TDM should be considered on day 1 to achieve the target AUC, especially with once-daily administration.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"244-251"},"PeriodicalIF":1.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142768688","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Assessment and management of pregnancy in patients with myeloproliferative neoplasms: insights from a single-institution study of 29 neonates. 骨髓增生性肿瘤患者妊娠的评估和管理:来自29名新生儿的单机构研究的见解。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-02-01 Epub Date: 2024-12-06 DOI: 10.1007/s12185-024-03893-y
Yoko Edahiro, Shuichi Shirane, Jun Takeda, Hajime Yasuda, Tadaaki Inano, Miyuki Tsutsui, Yasuharu Hamano, Makoto Sasaki, Jun Ando, Atsuo Itakura, Miki Ando, Norio Komatsu
{"title":"Assessment and management of pregnancy in patients with myeloproliferative neoplasms: insights from a single-institution study of 29 neonates.","authors":"Yoko Edahiro, Shuichi Shirane, Jun Takeda, Hajime Yasuda, Tadaaki Inano, Miyuki Tsutsui, Yasuharu Hamano, Makoto Sasaki, Jun Ando, Atsuo Itakura, Miki Ando, Norio Komatsu","doi":"10.1007/s12185-024-03893-y","DOIUrl":"10.1007/s12185-024-03893-y","url":null,"abstract":"","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"281-283"},"PeriodicalIF":1.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142785645","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Carcinocythemia diagnosed on peripheral blood clot sections. 通过外周血凝块切片诊断出癌细胞增多症。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-02-01 Epub Date: 2024-12-16 DOI: 10.1007/s12185-024-03899-6
Henry Wood, Olivia McKinney
{"title":"Carcinocythemia diagnosed on peripheral blood clot sections.","authors":"Henry Wood, Olivia McKinney","doi":"10.1007/s12185-024-03899-6","DOIUrl":"10.1007/s12185-024-03899-6","url":null,"abstract":"","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"149-150"},"PeriodicalIF":1.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142828267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Correction: JSH practical guidelines for hematological malignancies, 2023: I. Leukemia-1. Acute myeloid leukemia (AML). 修正:JSH恶性血液病实用指南,2023:I.白血病-1。急性髓性白血病(AML)。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-02-01 DOI: 10.1007/s12185-024-03907-9
Yoshinobu Maeda
{"title":"Correction: JSH practical guidelines for hematological malignancies, 2023: I. Leukemia-1. Acute myeloid leukemia (AML).","authors":"Yoshinobu Maeda","doi":"10.1007/s12185-024-03907-9","DOIUrl":"10.1007/s12185-024-03907-9","url":null,"abstract":"","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"286"},"PeriodicalIF":1.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142882047","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Establishment of a high-risk pediatric AML-derived cell line YCU-AML2 with genetic and metabolic vulnerabilities. 具有遗传和代谢脆弱性的高危儿童aml衍生细胞系YCU-AML2的建立
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-02-01 DOI: 10.1007/s12185-025-03929-x
Junji Ikeda, Norio Shiba, Shota Kato, Hiroyoshi Kunimoto, Yusuke Saito, Maiko Sagisaka, Mieko Ito, Hiroaki Goto, Yusuke Okuno, Wataru Nakamura, Masahiro Yoshitomi, Masanobu Takeuchi, Shuichi Ito, Hideaki Nakajima, Motohiro Kato, Shin-Ichi Tsujimoto
{"title":"Establishment of a high-risk pediatric AML-derived cell line YCU-AML2 with genetic and metabolic vulnerabilities.","authors":"Junji Ikeda, Norio Shiba, Shota Kato, Hiroyoshi Kunimoto, Yusuke Saito, Maiko Sagisaka, Mieko Ito, Hiroaki Goto, Yusuke Okuno, Wataru Nakamura, Masahiro Yoshitomi, Masanobu Takeuchi, Shuichi Ito, Hideaki Nakajima, Motohiro Kato, Shin-Ichi Tsujimoto","doi":"10.1007/s12185-025-03929-x","DOIUrl":"https://doi.org/10.1007/s12185-025-03929-x","url":null,"abstract":"<p><p>The prognosis of acute myeloid leukemia (AML) with KMT2A::MLLT3 rearrangement and MECOM overexpression and/or KRAS mutation is dismal, and the optimal treatment strategy remains unclear. However, to the best of our knowledge, a suitable model (such as a cell line or its xenograft model) for research on this subtype has not been established. We established a novel AML cell line, YCU-AML2, and its xenograft model harboring KMT2A::MLLT3 rearrangement, MECOM overexpression, and KRAS G12A mutation. YCU-AML2 xenograft mice models developed AML and mimicked the clinical phenotype of the original patient. YCU-AML2 expressed high sensitivity to MEK inhibitors, such as trametinib and selumetinib. Moreover, YCU-AML2 also exhibited high sensitivity to L-asparaginase with glutaminase activity, perhaps because of its reliance on oxidative phosphorylation via glutaminolysis as its main energy source. We believe that the YCU-AML2 cell line and its xenograft model can serve as models to explore the molecular pathogenesis of high-risk AML with KMT2A::MLLT3 rearrangement, MECOM overexpression, and/or KRAS mutation and develop new treatment strategies.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A case of autoimmune factor XIII deficiency due to clearance-accelerating and inhibitory anti-FXIII autoantibodies. 一例因清除加速性和抑制性抗 FXIII 自身抗体而导致的自身免疫性因子 XIII 缺乏症。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-02-01 Epub Date: 2024-11-26 DOI: 10.1007/s12185-024-03874-1
Hiroko Tsunemine, Masayoshi Souri, Wataru Kumode, Nobuyoshi Arima, Akitada Ichinose
{"title":"A case of autoimmune factor XIII deficiency due to clearance-accelerating and inhibitory anti-FXIII autoantibodies.","authors":"Hiroko Tsunemine, Masayoshi Souri, Wataru Kumode, Nobuyoshi Arima, Akitada Ichinose","doi":"10.1007/s12185-024-03874-1","DOIUrl":"10.1007/s12185-024-03874-1","url":null,"abstract":"<p><p>A 63-year-old man, previously diagnosed with multiple autoimmune diseases, developed life-threatening bleeding after gastrectomy for stomach cancer. He survived due to treatment with factor XIII (FXIII) concentrates immediately after his FXIII antigen (Ag) level was reported to be < 5% of normal. Detailed examination by the Japanese Collaborative Research Group on autoimmune coagulation factor deficiencies revealed the presence of anti-FXIII-A and anti-FXIII-B subunit autoantibodies on immunoblot analyses, and thus autoimmune FXIII deficiency (AiF13D) was diagnosed based on the Japanese and international diagnostic criteria. Antibody eradication therapy with prednisolone was initiated and cyclophosphamide was added later. While FXIII:Ag levels remained at 40-50% of normal, bleeding did not recur even after stomach polypectomy. Experimental studies on patient specimens collected at the initial bleeding and later asymptomatic stages demonstrated the co-existence of clearance-accelerating and inhibitory anti-FXIII autoantibodies. The former type was predominant in both the bleeding and asymptomatic stages, whereas the latter became distinct in the asymptomatic stage. This is the first AiF13D patient to demonstrate such a change in anti-FXIII autoantibody type during the clinical course. This report discusses the relationship between autoantibody type and bleeding phenotype in detail, but future large studies are needed to confirm these observations.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"257-264"},"PeriodicalIF":1.7,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142716154","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Chronic graft-versus-host disease myelitis successfully treated with rituximab. 利妥昔单抗成功治疗慢性移植物抗宿主病脊髓炎。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-01-31 DOI: 10.1007/s12185-025-03936-y
Emi Yokoyama, Yuta Hasegawa, Kentaro Wakaki, Touma Suzuki, Sayaka Kajikawa, Minoru Kanaya, Koh Izumiyama, Makoto Saito, Masanobu Morioka, Jun Nagai, Tomoe Ichiki, Ryo Kikuchi, Satomi Okada, Hiroyuki Ohigashi, Hideki Goto, Masahiro Onozawa, Daigo Hashimoto, Akio Mori, Takanori Teshima, Takeshi Kondo
{"title":"Chronic graft-versus-host disease myelitis successfully treated with rituximab.","authors":"Emi Yokoyama, Yuta Hasegawa, Kentaro Wakaki, Touma Suzuki, Sayaka Kajikawa, Minoru Kanaya, Koh Izumiyama, Makoto Saito, Masanobu Morioka, Jun Nagai, Tomoe Ichiki, Ryo Kikuchi, Satomi Okada, Hiroyuki Ohigashi, Hideki Goto, Masahiro Onozawa, Daigo Hashimoto, Akio Mori, Takanori Teshima, Takeshi Kondo","doi":"10.1007/s12185-025-03936-y","DOIUrl":"https://doi.org/10.1007/s12185-025-03936-y","url":null,"abstract":"<p><p>Chronic graft-versus-host disease (cGVHD) is a major serious complication after allogeneic stem-cell transplantation (allo-HSCT), and often mimics autoimmune diseases. Central nervous system (CNS) symptoms are rare manifestations of cGVHD, and are difficult to diagnose. CNS manifestations of cGVHD were discussed in the 2020 National Institutes of Health cGVHD Consensus Project as one of the \"atypical cGVHD manifestations\" with involvement of various organ systems other than classical cGVHD organs. We experienced a case of myelitis after allo-HSCT diagnosed as cGVHD of the CNS. The neurological symptoms progressed after corticosteroid pulse therapy, resulting in severe paralysis and paresthesia of the lower extremities. The clinical course and magnetic resonance imaging findings showed some similarities with multiple sclerosis. We decided to use rituximab after the patient became refractory to corticosteroids because rituximab has been reported to be effective in multiple sclerosis by suppressing B cells on both sides of the blood-brain barrier. Rituximab was effective for the neurologic symptoms in our case. In atypical cGVHD, treatments used in corresponding autoimmune diseases may be reasonable and effective.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143065523","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024: part 4-trauma, burn, obstetrics, acute pancreatitis/liver failure, and others. 日本2024弥散性血管内凝血管理临床实践指南:第4部分:创伤、烧伤、产科、急性胰腺炎/肝衰竭等。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-01-31 DOI: 10.1007/s12185-025-03918-0
Mineji Hayakawa, Yoshinobu Seki, Takayuki Ikezoe, Kazuma Yamakawa, Kohji Okamoto, Shigeki Kushimoto, Yuichiro Sakamoto, Yuki Itagaki, Yuki Takahashi, Hiroyasu Ishikura, Toshihiko Mayumi, Toshihisa Tamura, Kenji Nishio, Yu Kawazoe, Ayami Shigeno, Yudai Takatani, Akihito Tampo, Yoshihiko Nakamura, Katsunori Mochizuki, Noritaka Yada, Kaoru Kawasaki, Akira Kiyokawa, Mamoru Morikawa, Mitsuhiro Uchiba, Takeshi Matsumoto, Hidesaku Asakura, Seiji Madoiwa, Toshimasa Uchiyama, Shinya Yamada, Shin Koga, Takashi Ito, Toshiaki Iba, Noriaki Kawano, Satoshi Gando, Hideo Wada
{"title":"Clinical practice guidelines for management of disseminated intravascular coagulation in Japan 2024: part 4-trauma, burn, obstetrics, acute pancreatitis/liver failure, and others.","authors":"Mineji Hayakawa, Yoshinobu Seki, Takayuki Ikezoe, Kazuma Yamakawa, Kohji Okamoto, Shigeki Kushimoto, Yuichiro Sakamoto, Yuki Itagaki, Yuki Takahashi, Hiroyasu Ishikura, Toshihiko Mayumi, Toshihisa Tamura, Kenji Nishio, Yu Kawazoe, Ayami Shigeno, Yudai Takatani, Akihito Tampo, Yoshihiko Nakamura, Katsunori Mochizuki, Noritaka Yada, Kaoru Kawasaki, Akira Kiyokawa, Mamoru Morikawa, Mitsuhiro Uchiba, Takeshi Matsumoto, Hidesaku Asakura, Seiji Madoiwa, Toshimasa Uchiyama, Shinya Yamada, Shin Koga, Takashi Ito, Toshiaki Iba, Noriaki Kawano, Satoshi Gando, Hideo Wada","doi":"10.1007/s12185-025-03918-0","DOIUrl":"https://doi.org/10.1007/s12185-025-03918-0","url":null,"abstract":"<p><p>Disseminated intravascular coagulation (DIC) is a complex condition with diverse etiologies. While its association with sepsis has been widely studied, less focus has been given to DIC arising from other critical conditions, such as trauma, burns, acute pancreatitis, and obstetric complications. The 2024 Clinical Practice Guidelines, developed by the Japanese Society on Thrombosis and Hemostasis (JSTH), aim to fill this gap and offer comprehensive recommendations for managing DIC across various conditions. This study, Part 4 of the guideline series, addresses DIC management in trauma, burns, obstetric complications, acute pancreatitis/liver failure, viral infections, and autoimmune diseases. For trauma-associated DIC, early administration of fresh-frozen plasma (FFP), coagulation factor concentrates such as fibrinogen and prothrombin complex concentrates, and tranexamic acid is recommended. The guidelines also highlight DIC in obstetrics, which is associated with massive bleeding, and recommend the administration of fibrinogen concentrate, antithrombin concentrate, and tranexamic acid. Through a systematic review of the current evidence, the guidelines provide stratified recommendations aimed at improving clinical outcomes in DIC management beyond sepsis, thereby serving as a valuable resource for healthcare providers globally.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143074320","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Response to initial treatment with glucocorticoids in TAFRO syndrome and implications for secondary treatment. TAFRO综合征初始糖皮质激素治疗的反应及其对二次治疗的影响。
IF 1.7 4区 医学
International Journal of Hematology Pub Date : 2025-01-29 DOI: 10.1007/s12185-025-03933-1
Ryutaro Tominaga, Kento Umino, Seina Honda, Daizo Yokoyama, Atsuto Noguchi, Shuka Furuki, Shunsuke Koyama, Rui Murahashi, Hirotomo Nakashima, Kazuki Hyodo, Shin-Ichiro Kawaguchi, Yumiko Toda, Daisuke Minakata, Masahiro Ashizawa, Chihiro Yamamoto, Kaoru Hatano, Kazuya Sato, Ken Ohmine, Shin-Ichiro Fujiwara, Yoshinobu Kanda
{"title":"Response to initial treatment with glucocorticoids in TAFRO syndrome and implications for secondary treatment.","authors":"Ryutaro Tominaga, Kento Umino, Seina Honda, Daizo Yokoyama, Atsuto Noguchi, Shuka Furuki, Shunsuke Koyama, Rui Murahashi, Hirotomo Nakashima, Kazuki Hyodo, Shin-Ichiro Kawaguchi, Yumiko Toda, Daisuke Minakata, Masahiro Ashizawa, Chihiro Yamamoto, Kaoru Hatano, Kazuya Sato, Ken Ohmine, Shin-Ichiro Fujiwara, Yoshinobu Kanda","doi":"10.1007/s12185-025-03933-1","DOIUrl":"https://doi.org/10.1007/s12185-025-03933-1","url":null,"abstract":"<p><p>The study aimed to investigate the therapeutic effect of various initial treatments incorporating glucocorticoid (GC) in TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly). Cases of TAFRO syndrome up to November 2023 were retrospectively collected. Overall survival (OS) and resistance to GC therapy were assessed, with resistance analyzed based on the time to the next treatment or death (TTNTD). The study included 95 patients, including 5 diagnosed at our hospital. OS did not differ significantly between patients who received GC monotherapy and those who had a second-line therapy added within 2 weeks (100-day OS rate: 86.6% vs. 77.7%; p = 0.338). Moreover, 100-day OS did not differ between patients who received GC pulse therapy within 2 weeks and those who did not (77.5% vs. 93.1%, p = 0.129). In multivariate analyses, pretreatment severity score ≥ 8 (hazard ratio [HR], 2.99; 95% confidence interval [CI] 1.05-8.50) and platelets ≥ 6.9 × 10^4/µL (HR, 2.26; 95% CI 1.01-5.02) were significantly associated with shorter TTNTD. Additional second-line or GC pulse therapy provided no advantage in the hyperacute phase. Higher severity scores and platelet values may predict resistance to GC therapy.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143065525","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","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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