International Journal of Hematology最新文献

JSH practical guidelines for hematological malignancies, 2023: II. Lymphoma 4. Mantle cell lymphoma (MCL). 恶性血液病实用指南，2023:II。淋巴瘤4。套细胞淋巴瘤（MCL）。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-03-24 DOI: 10.1007/s12185-025-03960-y

Kenichi Ishizawa, Kazuhito Yamamoto

引用次数: 0

Efficacy and safety of the thiotepa-busulfan conditioning regimen as for autologous stem cell transplantation in relapsed/refractory systemic diffuse large B cell lymphoma: a single-center retrospective study. thiotepa-busulfan调节方案用于自体干细胞移植治疗复发/难治性全身弥漫性大B细胞淋巴瘤的有效性和安全性：一项单中心回顾性研究

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-02-12 DOI: 10.1007/s12185-025-03946-w

Katsuhiro Io, Kenichi Nagai, Bunta Kakihara, Kiyotaka Izumi, Tomoya Kitagawa

{"title":"Efficacy and safety of the thiotepa-busulfan conditioning regimen as for autologous stem cell transplantation in relapsed/refractory systemic diffuse large B cell lymphoma: a single-center retrospective study.","authors":"Katsuhiro Io, Kenichi Nagai, Bunta Kakihara, Kiyotaka Izumi, Tomoya Kitagawa","doi":"10.1007/s12185-025-03946-w","DOIUrl":"10.1007/s12185-025-03946-w","url":null,"abstract":"High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) is a standard treatment for relapsed diffuse large B cell lymphoma (DLBCL). The BEAM regimen is widely used, but the MEAM regimen is more common in Japan due to the unavailability of carmustine. This retrospective analysis evaluated the efficacy of the thiotepa and busulfan (TT/BU) regimen compared with other regimens in 27 patients with systemic DLBCL who underwent ASCT at our institution from December 2013 to March 2022. Fourteen patients received the TT/BU regimen, while 13 received alternative regimens. The TT/BU regimen demonstrated superior progression-free survival (PFS) and overall survival (OS) compared to other regimens, with a 3 year PFS of 84.4% and OS of 91.7%. The TT/BU group also had fewer severe adverse events, particularly regarding renal function. Our findings suggest that the TT/BU regimen is a well-tolerated and effective alternative for relapsed/refractory DLBCL and provide valuable insights for future treatment strategies.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"813-819"},"PeriodicalIF":1.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12106555/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143398988","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

Prognostic impact of effusion in multiple body cavities after allogeneic hematopoietic stem cell transplantation. 异基因造血干细胞移植后多体腔积液对预后的影响。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-03-03 DOI: 10.1007/s12185-025-03949-7

Yasutaka Masuda, Akira Honda, Takashi Oyama, Yosuke Masamoto, Mineo Kurokawa

{"title":"Prognostic impact of effusion in multiple body cavities after allogeneic hematopoietic stem cell transplantation.","authors":"Yasutaka Masuda, Akira Honda, Takashi Oyama, Yosuke Masamoto, Mineo Kurokawa","doi":"10.1007/s12185-025-03949-7","DOIUrl":"10.1007/s12185-025-03949-7","url":null,"abstract":"Fluid retention presenting as effusions in body cavities is sometimes encountered following allogeneic stem cell transplantation (allo-HSCT). It is unclear whether cavity effusions at independent sites may serve as cumulative correlates of fluid overload and whether a higher number of effusion sites are associated with a worse prognosis. Here, we comprehensively reviewed pleural, peritoneal, and pericardial effusions in 178 first allo-HSCT recipients retrospectively. A total of 123 (69.1%) patients developed effusions in any cavity. New pleural, peritoneal, and pericardial effusions were found after allo-HSCT in 106, 88, and 53 patients, at a median of 38.0 (range, 2-2950), 22.5 (range, 2-1324), and 40 (range, 2-945) days, respectively. The cumulative incidence at day 100 was 41.0%, 40.4%, and 20.8%, respectively. Of the 92 patients who presented with effusions by day 100, 28 patients presented with effusion in a single cavity, 39 in two cavities, and 25 in all three cavities. The 2-year overall survival rates of patients with effusions in zero, one, two, and three cavities by day 100 were 86.1%, 60.0%, 59.6%, and 18.8%, respectively, showing an additive adverse association with outcome. Prospective studies to further characterize fluid dynamics following allo-HSCT are warranted.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"833-847"},"PeriodicalIF":1.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12106147/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143541982","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

The novel protein C variant p.C101F results in early intracellular degradation that drives type I protein C deficiency. 新的蛋白C变体p.C101F导致细胞内早期降解，导致I型蛋白C缺乏。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-02-10 DOI: 10.1007/s12185-025-03943-z

Rikuto Yui, Satomi Nagaya, Ibuki Yasuda, Tomoki Togashi, Yuika Kikuchi, Kengo Saito, Makiko Meguro-Horike, Shin-Ichi Horike, Hiroshi Kawasaki, Hidekazu Nishikii, Eriko Morishita

{"title":"The novel protein C variant p.C101F results in early intracellular degradation that drives type I protein C deficiency.","authors":"Rikuto Yui, Satomi Nagaya, Ibuki Yasuda, Tomoki Togashi, Yuika Kikuchi, Kengo Saito, Makiko Meguro-Horike, Shin-Ichi Horike, Hiroshi Kawasaki, Hidekazu Nishikii, Eriko Morishita","doi":"10.1007/s12185-025-03943-z","DOIUrl":"10.1007/s12185-025-03943-z","url":null,"abstract":"Hereditary protein C (PC) deficiency is an inherited thrombophilic disorder caused by variants in the PC gene (PROC). We identified a novel PROC variant, c.302G>T, p.Cys101Phe (C101F), in a patient with type I PC deficiency. We analyzed the intracellular dynamics of the C101F variant of PC (PC-C101F) to elucidate the pathogenic mechanism underlying this condition. Wild-type PC (PC-WT) and PC-C101F were transiently expressed in HEK293 cells for expression and functional analyses. The PC antigen levels in the cell lysate and culture supernatant of PC-C101F-expressing cells were significantly lower than those of PC-WT-expressing cells. In cycloheximide (CHX) chase experiments, the intracellular PC antigen level gradually decreased in PC-C101F-expressing cells, but remained stable at 0 and 6 h in the presence of CHX/MG132. No significant difference in co-localization with the endoplasmic reticulum was observed between PC-C101F and PC-WT. 101Cys forms a disulfide bond with 106Cys, which is crucial for maintaining the conformation of PC. PC-C101F likely results in protein misfolding and proteasomal degradation, leading to type I PC deficiency. These findings highlight the importance of cysteine residues in the three-dimensional structure of PC and provide insight into the mechanism of type I PC deficiency.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"774-781"},"PeriodicalIF":1.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143382332","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

Disease response criteria in Langerhans cell histiocytosis: a global view. 朗格汉斯细胞组织细胞增多症的疾病反应标准：全局观点。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-04-27 DOI: 10.1007/s12185-025-03989-z

Ilia N Buhtoiarov, Milen Minkov, Reza Vali, Oussama Abla

{"title":"Disease response criteria in Langerhans cell histiocytosis: a global view.","authors":"Ilia N Buhtoiarov, Milen Minkov, Reza Vali, Oussama Abla","doi":"10.1007/s12185-025-03989-z","DOIUrl":"10.1007/s12185-025-03989-z","url":null,"abstract":"Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm with heterogeneous presentations. The discovery of BRAFV600E and other MAPK pathway mutations drastically transformed the treatment landscape, especially for high-risk LCH and CNS-LCH. While treatment strategies for children and adults are somewhat similar, response assessment methodologies remain highly dichotomized.Currently, separate treatment response criteria exist for children and adults, especially in therapeutic trials. Considering the rapid evolution of targeted MAPK-inhibitor therapies, along with ultrasensitive detection of minimal residual disease biomarkers (e.g., circulating BRAFV600E-encoding DNA) and sophisticated imaging tools (18F-FDG-PET and whole-body MRI), harmonization of response criteria in LCH is clearly warranted. The Histiocyte Society Global LCH Treatment Response Harmonization Task Force, a collaborative network of pediatric and adult LCH experts, is set to propose updated pediatric LCH treatment response criteria, which will also serve as the foundation for a universal response assessment tool for pediatric and adult LCH. In this review, we focus on the past, present, and likely future of response assessment in LCH patients, and discuss needs that remain unmet in the targeted therapy era.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"756-766"},"PeriodicalIF":1.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12106586/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143964443","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

Iron deficiency anemia following long-term eltrombopag treatment for aplastic anemia: a single-institution experience. 再生障碍性贫血长期电曲柏治疗后缺铁性贫血：单一机构经验。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-02-12 DOI: 10.1007/s12185-025-03940-2

Ryusuke Yamamoto, Nobuhiro Hiramoto, Yuya Nagai, Takayuki Ishikawa, Tadakazu Kondo

{"title":"Iron deficiency anemia following long-term eltrombopag treatment for aplastic anemia: a single-institution experience.","authors":"Ryusuke Yamamoto, Nobuhiro Hiramoto, Yuya Nagai, Takayuki Ishikawa, Tadakazu Kondo","doi":"10.1007/s12185-025-03940-2","DOIUrl":"10.1007/s12185-025-03940-2","url":null,"abstract":"Eltrombopag (EPAG), an oral thrombopoietin receptor agonist, has shown excellent efficacy in patients with aplastic anemia (AA) alone or in combination with immunosuppressive therapy. EPAG also has the unexpected ability to chelate polyvalent cations, including iron. However, the association between long-term EPAG use and iron deficiency anemia (IDA) remains unclear. To address this, we retrospectively evaluated the incidence and characteristics of EPAG-induced IDA (E-IDA) in patients with AA at our institution. Of the 36 patients with AA receiving EPAG, six (17%) developed E-IDA without evidence of bleeding, with a median onset of 1142.5 days (range, 389-1442 days) after EPAG administration. The cumulative dose of EPAG was significantly higher in patients with E-IDA than in those without E-IDA (P = 0.04). In 4 patients, E-IDA occurred after hemoglobin levels improved above 10 g/dl; this was considered recurrent anemia. In the other 2 patients, E-IDA occurred when hemoglobin levels remained below 10 g/dl; this was considered resistant anemia. After oral iron supplementation, all patients achieved hemoglobin levels higher than their peak levels prior to the onset of E-IDA. E-IDA should be considered when patients with AA treated with EPAG longer than 1 year develop recurrent or resistant anemia.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"767-773"},"PeriodicalIF":1.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143398990","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

Karyotype evolution of myelodysplastic syndrome and acute myeloid leukemia with TP53 mutations. 伴TP53突变的骨髓增生异常综合征和急性髓性白血病的核型进化。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-02-19 DOI: 10.1007/s12185-025-03938-w

Yosuke Matsumoto, Daishi Kato, Ayako Muramatsu, Mio Sugitani, Tsutomu Kobayashi, Toshiki Iwai, Minako Mori, Daisuke Motooka, Jun Nakata, Seishi Ogawa, Yasuhito Nannya, Hitoji Uchiyama

{"title":"Karyotype evolution of myelodysplastic syndrome and acute myeloid leukemia with TP53 mutations.","authors":"Yosuke Matsumoto, Daishi Kato, Ayako Muramatsu, Mio Sugitani, Tsutomu Kobayashi, Toshiki Iwai, Minako Mori, Daisuke Motooka, Jun Nakata, Seishi Ogawa, Yasuhito Nannya, Hitoji Uchiyama","doi":"10.1007/s12185-025-03938-w","DOIUrl":"10.1007/s12185-025-03938-w","url":null,"abstract":"To clarify karyotype evolution of myelodysplastic syndrome or acute myeloid leukemia with TP53 mutations (MDS/AML-TP53), we analyzed G-banding of bone marrow aspiration samples of eight patients with MDS/AML-TP53 and visualized the evolutions as phylogenetic trees. With very few exceptions, the initial roots of these trees and all branches longitudinally had -5/5q- and -7/7q- in common. Time series data of the karyotypes obtained in six patients showed highly complex karyotype evolutions, such as combined branched, linear, parallel, and macro-evolutions. In two patients, numerous branches appeared as the initial transformation. As for aneuploidy, chromosome loss was more common than chromosome gain as previously reported. Structural and numerical chromosomal abnormalities were often deleted as karyotype evolution progressed. Among these karyotype evolutions, loss of translocated chromosomes with break sites at or near the centromeres frequently caused monosomies of two chromosomes involved in the translocation. G-banding enables analysis and visualization of karyotype evolutions as phylogenetic trees because it offers the properties of both single-cell and whole-chromosome analysis. Our research has led us to propose G-banding as a new interpretation method for classical karyotype analysis.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"792-800"},"PeriodicalIF":1.7,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143448984","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

JSH practical guidelines for hematological malignancies, 2023: II. Lymphoma3. Lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia (LPL/WM). 恶性血液病实用指南，2023:II。Lymphoma3。淋巴浆细胞性淋巴瘤/Waldenström巨球蛋白血症（LPL/WM）。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-02-25 DOI: 10.1007/s12185-025-03948-8

Isao Yoshida

引用次数: 0

Late-onset radiation necrosis of the larynx attributed to hydroxyurea taken for essential thrombocythemia. 因服用羟基脲治疗原发性血小板增多症而导致晚期喉部放射性坏死。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2025-06-01 Epub Date: 2025-03-17 DOI: 10.1007/s12185-025-03974-6

Masahiro Tokunaga, Akiyuki Yamato, Hidenori Inohara, Tetsuo Maeda

引用次数: 0

Spherocytic hemolytic anemia caused by Clostridium perfringens. 由产气荚膜梭菌引起的球型溶血性贫血。