American Journal of Hematology最新文献

{"title":"Defining IgM Multiple Myeloma.","authors":"Mahin Bhatt, Anaelle Zimmowitch, Hadiyah Yasin Audil, S Vincent Rajkumar","doi":"10.1002/ajh.70097","DOIUrl":"https://doi.org/10.1002/ajh.70097","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":" ","pages":""},"PeriodicalIF":9.9,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145197909","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"GPVI as a Potential New Biomarker for Myelofibrosis Diagnosis","authors":"Xavier Hittinger, Yannick Le Bris, Yacine El Aiba, Sophie Vantyghem, Raphael Bourgade, Romain Madeuf, Antoine Bonnet, Marion Loirat, Bruno Villemagne, Patrice Chevallier, Antoine Babuty, Simon Bouzy, Marc Fouassier","doi":"10.1002/ajh.70066","DOIUrl":"https://doi.org/10.1002/ajh.70066","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"71 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145188461","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Facts and Misfacts on D-Dimer Testing. Consensus Guidance From the Italian Society on Thrombosis and Hemostasis (SISET).","authors":"Armando Tripodi,Walter Ageno,Luca Barcella,Antonio Coppola,Marcello Di Nisio,Massimo Franchini,Monia Marchetti,Rossella Marcucci,Marco Marietta,Pasquale Pignatelli,Domenico Prisco,Valerio De Stefano","doi":"10.1002/ajh.70079","DOIUrl":"https://doi.org/10.1002/ajh.70079","url":null,"abstract":"D-dimer defines degradation products derived from the proteolysis mediated by plasmin on cross-linked fibrin. The evidence-based use of D-dimer in some conditions has been consolidated. Currently, however, there is an entrenched prescription of D-dimer testing to screen otherwise healthy subjects that may induce prescribing physicians to start time-consuming diagnostic procedures that may generate patient anxiety/distress and often fail to reach meaningful conclusions for patients' management. To make things worse, the incidental finding of unexplained elevated D-dimer is often, and inadvertently, taken as an index of venous thromboembolism (VTE) that may induce unnecessary initiation of thromboprophylaxis. Finally, population studies showed that elevated D-dimer is variably associated with arterial thrombosis. However, the above studies do not allow establishing a direct causal relationship between elevated D-dimer and disease, which makes D-dimer measurement hardly applicable in individual patients. With this as background, the Italian Society on Thrombosis and Hemostasis appointed a panel to prepare a guidance on the use of D-dimer based on the literature and expert opinion. The main conclusions of the panel can be summarized as follows: (i) D-dimer can be reliably used in combination with pre-test clinical probability to exclude VTE in symptomatic outpatients. (ii) It may help establish the optimal duration of secondary VTE prevention with vitamin K antagonists, although the panel felt that this area needs further investigation when dealing with direct oral anticoagulants. (iv) The use of D-dimer in combination with the pre-test probability score can be used to safely rule out acute aortic syndromes in the emergency setting without further imaging tests.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"7 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145182559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Venetoclax in Combination With Pediatric-Inspired Chemotherapy in Adults With Newly Diagnosed Acute Lymphoblastic Leukemia: Results of a Phase I Trial","authors":"Yannis K. Valtis, David Nemirovsky, Andriy Derkach, Isabella Cacace, Martina Torres, Colleen Finneran, Madhulika Shukla, Aaron D. Goldberg, Eytan M. Stein, Mark B. Geyer, Jae H. Park","doi":"10.1002/ajh.70082","DOIUrl":"https://doi.org/10.1002/ajh.70082","url":null,"abstract":"<p>While the outcomes of acute lymphoblastic leukemia (ALL) treatment in young children are excellent with > 90% long-term event-free survival (EFS) [<span>1</span>], outcomes among adults have continued to lag behind. Even with the utilization of pediatric-inspired chemotherapy (PIC) regimens, there remains a significant unmet need for 25%–40% of adult ALL patients who are not cured with upfront chemotherapy [<span>2, 3</span>]. Venetoclax is a B-cell lymphoma 2 (BCL-2) inhibitor approved for the treatment of acute myeloid leukemia (AML) and chronic lymphocytic leukemia (CLL) in combination with other agents. Preclinical data has long indicated that ALL blasts express high levels of BCL-2 and are sensitive to its inhibition [<span>4</span>]. Based on this data, several clinical trials have incorporated venetoclax in combination with different chemotherapy regimens for relapsed/refractory (R/R) ALL [<span>5-7</span>] as well as in upfront treatment [<span>8</span>] with encouraging results. These reports prompted the question of whether venetoclax can be safely added to a PIC regimen and whether that approach can improve rates of measurable residual disease-negative complete remission (MRD-CR) rates compared to historical treatments.</p>\u0000<p>We conducted a phase I clinical trial of oral venetoclax in combination with asparaginase-containing PIC in adult patients with newly diagnosed ALL (NCT05386576). The study was approved by the MSKCC Institutional Review Board and conducted in accordance with the Declaration of Helsinki. The study design was a 3 + 3 dose de-escalation design with 2 dose levels. The total study accrual goal was 12 patients. Inclusion criteria included age 18–60 at the time of registration, ECOG performance status 0–2, and adequate organ function. Further details on the study design, definitions of dose limiting toxicities, and treatment plan are provided in the accompanying appendix.</p>\u0000<p>The chemotherapy backbone regimen used in this trial is identical to the one utilized in the published MSKCC PIC protocol (Figure S1) [<span>3</span>]. Venetoclax was administered in the following fashion in the initial protocol: During Induction 1, 100 mg on day 5, 200 mg on day 6, and 400 mg on days 7–28. In patients with Grade 4 neutropenia (Absolute Neutrophil Count (ANC) < 500/μL) with fever or Grade 4 thrombocytopenia (platelet count < 25 000/μL) after day 14, venetoclax was held until ANC recovered to ≥ 1000/μL and platelets recovered to ≥ 50 000/μL. Patients proceeded from Induction I to Induction II on day 35–43 regardless of count recovery, unless the patient demonstrated marrow aplasia in two subsequent biopsies, in which case they were removed from the study (this did not occur to any patient). During Induction II, venetoclax was given at 400 mg during days 1–14 and 29–42. In patients with ANC < 500/μL with fever, ANC < 100/μL irrespective of fever, or platelet < 25 000/μL after day 29, venetoclax was held until ","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"101 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145153623","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence and Risk Factors for Venous Thromboembolism in People With Inherited Bleeding Disorders: An ATHNdataset Study.","authors":"Shreya Agarwal,Joseph R Stanek,Jianzhong Hu,Tammuella Christentry-Singleton,Vilmarie Rodriguez","doi":"10.1002/ajh.70092","DOIUrl":"https://doi.org/10.1002/ajh.70092","url":null,"abstract":"It is presumed that people with inherited bleeding disorders are protected against the risk of venous thromboembolism (VTE) but the supporting data regarding rates and risk factors for VTE in this population are lacking. The objectives of this study were to determine the VTE rates and risk factors in a cohort of individuals with inherited bleeding disorders. American Thrombosis and Hemostasis Network (ATHN) dataset was utilized for this study. The population of interest was individuals with an inherited bleeding disorder with a documented VTE. Data were collected regarding their bleeding disorder, demographics, type of VTE, and comorbidities. Data were summarized using standard descriptive analyses, and logistic regression analyses were used to identify risk factors. We identified a total of 49,151 individuals with inherited bleeding disorders in the ATHNdataset. A total of 388 new VTE (0.8%; 95% CI: 0.7, 0.9) were observed. Median age at VTE diagnosis was 27.5 years (IQR: 15.3-48.2). VTE rates were highest in prothrombin deficiency at 19% and relatively lower in hemophilia A and B at 0.4%. Within the hemophilia cohort, rates of VTE were higher in those with inhibitors (p < 0.001). The VTE recurrence rate was 4.9% (n = 19/388). On multivariable analysis, Black race (OR = 1.90), central venous line (CVL) (OR = 2.76), and cardiac comorbidity (OR = 16.86) were identified as risk factors for VTE. In summary, our results demonstrate that people with inherited bleeding disorders are not protected against VTE. Individual factors such as cardiac comorbidity and the presence of a CVL can increase the prothrombotic risk.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"18 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145153360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk Factors for and Impact of Pre-Engraftment Syndrome on Outcomes Following Single-Unit Cord Blood Transplantation in Adults.","authors":"Masatoshi Sakurai,Keisuke Kataoka,Kota Mizuno,Takuto Mori,Shuichi Shirane,Hirotoshi Sakaguchi,Takehiko Mori,Masatsugu Tanaka,Masahito Tokunaga,Makoto Onizuka,Mamiko Sakata-Yanagimoto,Jun Ishikawa,Yuta Katayama,Shuichi Ota,Masashi Sawa,Jun Kato,Yuta Hasegawa,Koichi Onodera,Norimichi Hattori,Shigesaburo Miyakoshi,Nobuyuki Takayama,Tetsuya Nishida,Koji Kato,Fumihiko Ishimaru,Yoshiko Atsuta,Junya Kanda,Hideki Nakasone,Seitaro Terakura","doi":"10.1002/ajh.70094","DOIUrl":"https://doi.org/10.1002/ajh.70094","url":null,"abstract":"Pre-engraftment syndrome (PES) is a unique complication of cord blood transplantation (CBT) whose risk factors and impact on transplant outcomes remain controversial. Using a nationwide database in Japan, we analyzed a total of 3734 patients who underwent single-unit CBT. PES occurred in 18.3% of patients, and risk factors for PES included a higher hematopoietic cell transplantation-specific comorbidity index, first transplantation, myeloablative conditioning (MAC), lower total nucleated cell (TNC) dose, and graft-versus-host disease (GVHD) prophylaxis regimens excluding tacrolimus with methotrexate. Patients who developed PES had significantly higher incidences of grade II-IV acute GVHD (53.1% vs. 31.3%, p < 0.001) and chronic GVHD (27.2% vs. 21.7%, p = 0.002) compared to those without PES. Landmark analysis with multivariable adjustment revealed that PES was independently associated with increased non-relapse mortality (NRM, hazard ratio [HR] 1.46; 95% CI 1.22-1.75; p < 0.001), reduced relapse incidence (HR 0.78; 95% CI 0.63-0.96; p = 0.020), and a trend toward inferior overall survival (OS, HR 1.13; 95% CI 0.98-1.30; p = 0.088). Moreover, patients who experienced both PES and acute GVHD had the highest 2-year NRM (31.7%; 95% CI 26.0%-37.6%) and the lowest 2-year OS (55.9%; 95% CI 50.0%-62.4%), compared with those who experienced either PES (NRM 20.7%; OS 65.0%) or acute GVHD (NRM 19.5%; OS 62.8%) (p < 0.001), highlighting the combined effects of these complications. This study, the largest to date on PES, demonstrates its clinical significance as an early complication with lasting effects on transplant outcomes.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"15 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145140507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ferritin, C-Reactive Protein, and Soluble CD25 Distinguish TAFRO From HLH.","authors":"Steven Rowe,Mariam Goubran,Mateo Sarmiento Bustamante,Saishravan Shyamsundar,Bridget Austin,Kathleen McNicholas,Marley Blommers,Junyan Shi,Lisa K Peterson,Lusia Sepiashvili,Andre Mattman,Joshua D Brandstadter,David C Fajgenbaum,Luke Y C Chen","doi":"10.1002/ajh.70081","DOIUrl":"https://doi.org/10.1002/ajh.70081","url":null,"abstract":"A clinical pathway for differentiating TAFRO from HLH when a cytokine storm is identified, using ferritin, C-reactive protein, and soluble CD25.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"24 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145140491","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessment of Systematic JAK2V617F Screening in 996 Patients With Venous Thromboembolism.","authors":"Norman Abbou,Julie Quessada,Antoine Tichadou,Robin Arcani,Régis Costello,Jean Gabert,Pierre-Emmanuel Morange,Pierre Suchon,Geoffroy Venton","doi":"10.1002/ajh.70095","DOIUrl":"https://doi.org/10.1002/ajh.70095","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"126 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145134005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Validation of BLAST and BLAST-Mol Risk Models in Chronic Myelomonocytic Leukemia: Mayo-Humanitas Collaborative Project Involving 1101 Patients.","authors":"Saubia Fathima,Ali Alsugair,Muhammad Yousuf,Priyansh Faldu,Clifford Csizmar,Merry Nakhleh,Abhishek A Mangaonkar,Animesh Pardanani,Luca Lanino,Alessia Campagna,Giulia Maggioni,Kaaren K Reichard,Rong He,Naseema Gangat,Mrinal M Patnaik,Matteo G Della Porta,Ayalew Tefferi","doi":"10.1002/ajh.70080","DOIUrl":"https://doi.org/10.1002/ajh.70080","url":null,"abstract":"The current study validates the predictive performance of the BLAST and BLAST-Mol risk models in a large two-center international cohort with a total of 1101 patients with newly diagnosed CMML.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"59 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145134033","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Early Detection of Diastolic Dysfunction in Sickle Cell Anemia: Can It Help the Patient and Let Us See the Elephant in the Room?","authors":"Thomas D Coates","doi":"10.1002/ajh.70073","DOIUrl":"https://doi.org/10.1002/ajh.70073","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"86 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145127341","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}