{"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}
{"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}
{"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}
{"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}
{"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}
{"title":"Correction: Treatment trends and risks of corticosteroid use in adult primary immune thrombocytopenia: a claims database study in Japan.","authors":"Hirokazu Kashiwagi, Isao Miura, Naohiko Terasawa, Ken-Ichi Iwayama, Yuka Furukawa, Makoto Kanenishi","doi":"10.1007/s12185-025-03931-3","DOIUrl":"10.1007/s12185-025-03931-3","url":null,"abstract":"","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":"143065524","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}
{"title":"Steroid-resistant nephrotic syndrome due to renal-limited thrombotic microangiopathy and membranous nephropathy after allogeneic hematopoietic stem cell transplantation successfully treated with calcineurin inhibitors.","authors":"Shinri Okada, Masashi Nishikubo, Yoshimitsu Shimomura, Nobuhiro Hiramoto, Keisuke Osaki, Shigeo Hara, Tadakazu Kondo, Takayuki Ishikawa","doi":"10.1007/s12185-025-03930-4","DOIUrl":"https://doi.org/10.1007/s12185-025-03930-4","url":null,"abstract":"<p><p>Transplantation-associated thrombotic microangiopathy (TMA) is a severe complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with high mortality. As calcineurin inhibitors (CNIs) reportedly contribute to TMA via drug-induced endothelial injury, treatment of TMA often involves CNI discontinuation or dose reduction. However, renal-limited TMA, defined as biopsy-proven renal TMA without the classical triad (hemolytic anemia, thrombocytopenia, and organ damage), has rarely been reported after allo-HSCT, and its optimal management remains unknown. Herein, we report three cases of renal-limited TMA after allo-HSCT that presented with nephrotic syndrome, in which renal biopsy showed TMA and concurrent membranous nephropathy. All patients were refractory to glucocorticoid monotherapy and the addition of CNIs led to complete remission of nephrotic syndrome. Renal-limited TMA after allo-HSCT may present as nephrotic syndrome with distinct pathophysiological features from renal-limited TMA in non-allo-HSCT recipients. Previous reports have suggested that renal-limited TMA after allo-HSCT is associated with renal graft-versus-host disease, and thus optimizing immunosuppressive therapy, including CNI treatment, may be useful. CNI treatment may be an option even in the presence of renal-limited TMA after allo-HSCT accompanied by concurrent membranous nephropathy.</p>","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":1.7,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143065526","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}
{"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}