International Journal of Hematology最新文献_第10页

Risk factors and remaining challenges in the treatment of acute promyelocytic leukemia. 急性早幼粒细胞白血病的风险因素和治疗中仍面临的挑战。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-11-01 Epub Date: 2024-02-22 DOI: 10.1007/s12185-023-03696-7

Yasuhisa Yokoyama

引用次数: 0

Relationship between hemoglobinopathies and male infertility: a scoping review. 血红蛋白病与男性不育之间的关系：范围综述。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-11-01 Epub Date: 2024-09-27 DOI: 10.1007/s12185-024-03844-7

Abdullah M Al-Jubouri, Ahmed Eliwa, Yunes Haithm, Noof Al-Qahtani, Lolwa Jolo, Mohamed Yassin

{"title":"Relationship between hemoglobinopathies and male infertility: a scoping review.","authors":"Abdullah M Al-Jubouri, Ahmed Eliwa, Yunes Haithm, Noof Al-Qahtani, Lolwa Jolo, Mohamed Yassin","doi":"10.1007/s12185-024-03844-7","DOIUrl":"10.1007/s12185-024-03844-7","url":null,"abstract":"Infertility is a common issue that threatens couples worldwide. Infertility can result from the male or female partner alone, or both partners. It can be due to multiple factors related to the patient's overall health or lifestyle. Causes related to patient health can be systemic or related to gonadal dysfunction. One of the systematic causes can be hematological. The two most common hemoglobinopathies that are thought to cause infertility, especially male infertility, are sickle cell disease (SCD) and thalassemia major (TM). These two hemoglobinopathies cause male infertility through pathophysiological alterations. Specifically, they alter the oxygen carrying ability of red blood cells (RBCs), causing tissue hypoxia that affects the normal physiological process of spermatogenesis, eventually inducing infertility. Semen analyses and other systemic blood testing can be used to investigate male infertility. Both hemoglobinopathies can be helped by blood transfusions, which can then alleviate male infertility. This paper aims to explore the relationship between hemoglobinopathies (SCD and TM) and their role in contributing to male infertility, in addition to the role of blood transfusions in addressing male infertility by correcting the root cause.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"566-574"},"PeriodicalIF":1.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11513741/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142346189","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

Recent advances in AML with mutated NPM1. 突变 NPM1 的急性髓细胞性白血病的最新进展。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-11-01 Epub Date: 2024-08-22 DOI: 10.1007/s12185-024-03835-8

Yuichi Ishikawa, Yoko Ushijima, Hitoshi Kiyoi

{"title":"Recent advances in AML with mutated NPM1.","authors":"Yuichi Ishikawa, Yoko Ushijima, Hitoshi Kiyoi","doi":"10.1007/s12185-024-03835-8","DOIUrl":"10.1007/s12185-024-03835-8","url":null,"abstract":"Nucleophosmin 1 (NPM1) mutation is one of the most prevalent genetic mutations in adult acute myeloid leukemia (AML) and is particularly predominant in AML with a normal karyotype. NPM1 is a chaperone protein that plays various roles in several cellular processes. Wild-type NPM1 is normally localized to the nucleus, whereas mutant NPM1 proteins exhibit altered cytoplasmic localization. Clinically, AML with mutated NPM1 without FLT3-ITD is associated with a higher complete remission rate and improved overall survival. AML with mutated NPM1 is categorized as a distinct genetic entity in the World Health Organization classification of hematopoietic malignancies due to its unique clinical and biological features. However, the precise roles of NPM1 in normal hematopoiesis and in AML development remain unclear. Recent studies have revealed various clinical applications of NPM1 mutations in AML treatment, particularly in measurable residual disease analyses that target mutant NPM1 transcripts and in potential therapeutic applications of menin inhibitors and XPO-1 inhibitors for AML with mutated NPM1. Thus, NPM1 mutation is highly significant in AML classification, prognosis, response assessment, and molecular targeted therapies. Here, we review recent progress in clinical and biological aspects of AML with mutated NPM1 including molecular targeted therapy.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"556-565"},"PeriodicalIF":1.7,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142035814","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

Metabolic dependencies of acute myeloid leukemia stem cells. 急性髓性白血病干细胞的代谢依赖性

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-10-01 Epub Date: 2024-05-15 DOI: 10.1007/s12185-024-03789-x

Xiangguo Shi, Mengdie Feng, Daisuke Nakada

引用次数: 0

Efficacy and safety of recombinant porcine factor VIII in Japanese patients with acquired hemophilia A. 重组猪因子 VIII 对日本获得性 A 型血友病患者的疗效和安全性。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-10-01 Epub Date: 2024-08-19 DOI: 10.1007/s12185-024-03823-y

Yoshinobu Seki, Yoshiyuki Ogawa, Takahide Kikuchi, Emiko Sakaida, Yuki Mizuta, Tadayuki Kitagawa, Kazuhiko Takemura, Yasuo Miyaguchi, Keiji Nogami, Tadashi Matsushita

{"title":"Efficacy and safety of recombinant porcine factor VIII in Japanese patients with acquired hemophilia A.","authors":"Yoshinobu Seki, Yoshiyuki Ogawa, Takahide Kikuchi, Emiko Sakaida, Yuki Mizuta, Tadayuki Kitagawa, Kazuhiko Takemura, Yasuo Miyaguchi, Keiji Nogami, Tadashi Matsushita","doi":"10.1007/s12185-024-03823-y","DOIUrl":"10.1007/s12185-024-03823-y","url":null,"abstract":"Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies inhibiting human factor VIII (hFVIII). This phase II/III open-label study evaluated the safety and efficacy of recombinant porcine factor VIII (rpFVIII, susoctocog alfa) in adults with AHA and severe bleeding episodes in Japan (NCT04580407). The initial rpFVIII dose was 200 U/kg, with subsequent doses based on clinical measures including plasma FVIII activity. The primary efficacy endpoint was the proportion of severe bleeding episodes with a positive response to rpFVIII therapy 24 h after treatment initiation. Five patients were eligible for, and completed, rpFVIII treatment (age group: 60s-80s; median hFVIII inhibitor: 52 BU/mL; porcine FVIII [pFVIII] inhibitor: 3/5 patients). The median (range) total dose/patient was 548.4 (198-1803) U/kg with a median 3.0 infusions/patient. All patients responded positively to rpFVIII therapy at 24 h regardless of baseline pFVIII inhibitor status. rpFVIII treatment was well tolerated with no adverse events of special interest such as thromboembolic events or de novo pFVIII inhibitors. This study supports the use of rpFVIII as a novel therapy in the clinical management of patients with AHA in Japan. rpFVIII was approved for treating bleeding episodes in adults with AHA in Japan in 2024.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"482-491"},"PeriodicalIF":1.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11415465/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141999848","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

Advances in pathogenesis research and challenges in treatment development for acute myeloid leukemia. 急性髓性白血病发病机制研究进展和治疗方法开发挑战。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-10-01 Epub Date: 2024-09-03 DOI: 10.1007/s12185-024-03837-6

Hiroki Yamaguchi

{"title":"Advances in pathogenesis research and challenges in treatment development for acute myeloid leukemia.","authors":"Hiroki Yamaguchi","doi":"10.1007/s12185-024-03837-6","DOIUrl":"10.1007/s12185-024-03837-6","url":null,"abstract":"Acute myeloid leukemia (AML) develops when hematopoietic stem cells acquire chromosomal and genetic abnormalities, transforming into leukemia stem cells (LSCs) and further gaining driver mutations. Advances in genomic analysis have identified numerous new gene mutations involved in AML development. Recent research has shown that individuals with germline mutations in genes like DDX41 and CEBPA develop AML upon acquiring additional somatic mutations, and the latest WHO classification separates AML with such mutations into distinct disease groups. LSCs are regulated by different metabolic processes than normal stem cells, contributing to drug resistance and relapse. LSCs rely on oxidative phosphorylation (OXPHOS) metabolism for energy production, and venetoclax inhibits this process, affecting LSCs. Resistant LSCs show enhanced glycolysis, which suggests that targeting both OXPHOS and glycolysis is crucial. While targeted therapies like FLT3, BCL-2, and IDH inhibitors have shown efficacy, resistance remains an issue, highlighting the need for new treatment strategies. CAR-T cell therapy is an emerging immunotherapy that shows particular promise for targeting CD123 and CLL-1, with acceptable toxicity. Future developments in CAR-T cell therapy and other immunotherapies are anticipated to improve AML treatment outcomes.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"414-416"},"PeriodicalIF":1.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142119763","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 impact of airflow obstruction after allogeneic hematopoietic stem cell transplantation. 异体造血干细胞移植后气流阻塞的临床影响。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-10-01 Epub Date: 2024-08-27 DOI: 10.1007/s12185-024-03831-y

Sanshiro Nakao, Shokichi Tsukamoto, Yusuke Takeda, Chikako Ohwada, Chihiro Ri, Shintaro Izumi, Yuri Kamata, Shinichiro Matsui, Asuka Shibamiya, Arata Ishii, Koji Takaishi, Kohei Takahashi, Yuki Shiko, Nagisa Oshima-Hasegawa, Tomoya Muto, Naoya Mimura, Koutaro Yokote, Chiaki Nakaseko, Emiko Sakaida

{"title":"Clinical impact of airflow obstruction after allogeneic hematopoietic stem cell transplantation.","authors":"Sanshiro Nakao, Shokichi Tsukamoto, Yusuke Takeda, Chikako Ohwada, Chihiro Ri, Shintaro Izumi, Yuri Kamata, Shinichiro Matsui, Asuka Shibamiya, Arata Ishii, Koji Takaishi, Kohei Takahashi, Yuki Shiko, Nagisa Oshima-Hasegawa, Tomoya Muto, Naoya Mimura, Koutaro Yokote, Chiaki Nakaseko, Emiko Sakaida","doi":"10.1007/s12185-024-03831-y","DOIUrl":"10.1007/s12185-024-03831-y","url":null,"abstract":"Criteria for airflow obstruction (AFO) at one year after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in pulmonary function tests (PFTs) are more stringent than the bronchiolitis obliterans syndrome (BOS) criteria of the National Institutes of Health. This single-center, retrospective cohort study evaluated the clinical impact of the AFO criteria at any time after transplantation. In 132 patients who underwent allo-HSCT from 2006 to 2016, the 2-year cumulative incidence of AFO was 35.0%, and the median time to diagnosis of AFO was 101 days after transplantation (range 35-716 days). Overall chronic graft-versus-host disease (cGVHD) incidence was significantly higher in patients with AFO than in those without AFO (80.4% vs. 47.7%, P < 0.01); notably, 37.0% of patients with AFO developed cGVHD after AFO diagnosis. AFO patients developed BOS with a 5-year cumulative incidence of 49.1% after AFO onset. The 5-year cumulative incidence of non-relapse mortality in the AFO group was higher than that in the non-AFO group (24.7% vs. 7.1%, P < 0.01). These results suggest that closely monitoring PFTs within two years after allo-HSCT, regardless of cGVHD status, is important for early detection of AFO and prevention of progression to BOS. (192words).","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"501-511"},"PeriodicalIF":1.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11415471/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142072782","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

Detection of factor XIII inhibitors in 33 patients with autoimmune factor XIII deficiency in Japan. 在日本 33 名自身免疫性因子 XIII 缺乏症患者中检测到因子 XIII 抑制剂。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-10-01 Epub Date: 2024-06-19 DOI: 10.1007/s12185-024-03807-y

Masayoshi Souri, Tsukasa Osaki, Akitada Ichinose

{"title":"Detection of factor XIII inhibitors in 33 patients with autoimmune factor XIII deficiency in Japan.","authors":"Masayoshi Souri, Tsukasa Osaki, Akitada Ichinose","doi":"10.1007/s12185-024-03807-y","DOIUrl":"10.1007/s12185-024-03807-y","url":null,"abstract":"Autoimmune factor XIII (FXIII) deficiency (AiF13D) is a rare hemorrhagic disease. The anti-FXIII autoantibodies that cause this disease are classified into three types: type Aa inhibits the heterotetramer assembly and activation of FXIII, type Ab inhibits the enzymatic activity of activated FXIII, and type B enhances the elimination of FXIII from the blood. The former two are FXIII inhibitors and may be lethal if overlooked by conventional functional assays. To reliably detect both types of FXIII inhibitors, a new assay was developed by incorporating 5-(biotinamido)pentylamine (BAPA) into α2-plasmin inhibitor (PI-BAPA assay). This assay was tested on plasma samples from 128 participants, including 60 healthy controls, 35 patients with non-immune acquired FXIII deficiency, and 33 patients with AiF13D (29 with type Aa inhibitors and 4 with type Ab inhibitors). The PI-BAPA assay successfully detected type Aa and Ab inhibitors in 5-step dilution cross-mixing tests between patient and normal plasma. This assay also showed comparable or superior inhibition rates in the 1:1 mixing test compared to conventional ammonia release and amine incorporation assays. Receiver operating characteristic curve analysis confirmed the excellent specificity and sensitivity of this assay for determining inhibition rates, and the assay has already been used for AiF13D diagnosis.","PeriodicalId":13992,"journal":{"name":"International Journal of Hematology","volume":" ","pages":"472-481"},"PeriodicalIF":1.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141418807","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

Immunohistochemical differentiation of extramedullary acute myeloid leukemia from blastic plasmacytoid dendritic cell neoplasm. 髓外急性髓细胞白血病与疱性浆细胞树突状细胞瘤的免疫组化鉴别。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-10-01 Epub Date: 2024-07-10 DOI: 10.1007/s12185-024-03817-w

Shuhei Kurosawa, Tomonori Nakazato

引用次数: 0

Achievement of deep molecular response and treatment-free remission with asciminib treatment in CML. 阿西米尼治疗 CML 可获得深度分子反应和无治疗缓解。

IF 1.7 4区医学

International Journal of Hematology Pub Date : 2024-10-01 Epub Date: 2024-07-18 DOI: 10.1007/s12185-024-03816-x

Ryo Yoshimaru, Yosuke Minami

引用次数: 0