American Journal of Hematology最新文献

{"title":"Corrigendum to \"Pyruvate Kinase Function Correlates With Red Blood Cell Properties and Clinical Manifestations in Sickle Cell Disease\".","authors":"","doi":"10.1002/ajh.27713","DOIUrl":"https://doi.org/10.1002/ajh.27713","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"88 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144103684","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":"Efficacy and Safety of the Bruton's Tyrosine Kinase Inhibitor Zanubrutinib in Immune Thrombocytopenia","authors":"Qiu-Sha Huang, Hai-Xia Fu, Chen-Cong Wang, Xiao-Lu Zhu, Yun He, Jin Wu, Qi Chen, Peng Zhao, Zhuo-Yu An, Kai-Yan Liu, Xiao-Jun Huang, Xiao-Hui Zhang","doi":"10.1002/ajh.27718","DOIUrl":"https://doi.org/10.1002/ajh.27718","url":null,"abstract":"Immune thrombocytopenia (ITP) is characterized by impaired platelet production and increased platelet destruction. Zanubrutinib is a highly selective next-generation Bruton tyrosine kinase (BTK) inhibitor that may reduce autoantibody production and reduce macrophage Fcγ receptor-mediated platelet destruction. In this single-arm, phase II study, we aimed to assess the efficacy and safety of zanubrutinib in corticosteroid-resistant or relapsed ITP. All patients received 80 mg zanubrutinib once daily for 6 weeks followed by a 20-week safety follow-up period. The primary endpoint was overall response (OR), defined as at least two consecutive platelet counts of at least 30 × 10⁹/L, at least a 2-fold increase in the baseline count, the absence of bleeding, and no need for rescue therapy at 4 weeks. The trial was registered with ClinicalTrials.gov, number NCT05279872. Between January 1, 2022 and October 30, 2022, 20 patients were enrolled. The median platelet count was 19 (10–25) × 10⁹/L at the time of enrollment. Participants had received a median of 4 (3–6) different therapies for ITP. Eleven (55%, 95% CI: 31.5%–76.9%) patients achieved an OR to the intervention. Two (10%) patients achieved a complete response. At the 6-month follow-up, a sustained response was achieved in seven (35.0%, 95% CI: 15.4%–59.2%) patients. There were no grade 4 or worse adverse events or treatment-related deaths. The most common adverse events were upper respiratory tract infection (in 25% of the patients). Zanubrutinib showed an encouraging response rate and tolerability, supporting its therapeutic potential for the treatment of ITP.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"18 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144097668","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":"Mixed Autoimmune Hemolytic Anemia: A Systematic Review of Epidemiology, Clinical Characteristics, Therapies, and Outcomes","authors":"Jeremy W. Jacobs, Sheharyar Raza, Landon M. Clark, Laura D. Stephens, Elizabeth S. Allen, Jennifer S. Woo, Rachel Lane Walden, Cristina A. Figueroa Villalba, Christopher A. Tormey, Caroline G. Stanek, Brian D. Adkins, Evan M. Bloch, Garrett S. Booth","doi":"10.1002/ajh.27721","DOIUrl":"https://doi.org/10.1002/ajh.27721","url":null,"abstract":"Mixed autoimmune hemolytic anemia (AIHA) is a rare and clinically complex hematologic disorder defined by the simultaneous presence of both warm and cold autoantibodies, resulting in severe and often treatment-resistant hemolysis. Due to variability in diagnostic criteria and limited data, a comprehensive understanding of its epidemiology, clinical characteristics, and management remains incomplete. To address these gaps, we performed a systematic literature review employing stringent diagnostic criteria to evaluate epidemiologic patterns, clinical features, and therapeutic outcomes. Our analysis included 81 patients identified across 35 studies, revealing a median age of 45 years and a notable female predominance (2.25:1). Autoimmune diseases constituted the most frequent underlying etiology, followed by hematologic malignancies and infections. Patients exhibited significant anemia, with median nadir hemoglobin levels reaching 5.6 g/dL. Corticosteroids represented the most common therapeutic intervention; however, only 43% of patients achieved remission, while 37% experienced chronic hemolysis, and mortality reached 11%. Many patients required multiple lines of therapy, including rituximab and cytotoxic agents, highlighting the disease's refractory nature and management complexity. The substantial variability in diagnostic and therapeutic approaches emphasizes an urgent need for standardized diagnostic criteria, earlier integration of combination therapies, and exploration of innovative treatment modalities. Future prospective, multicenter studies are essential to refine disease recognition, optimize therapeutic strategies, and ultimately improve patient outcomes in mixed AIHA.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"18 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144097579","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":"Watching the Barn Door Open—Timing CML Stem Cell Allografts","authors":"Jeffrey H. Lipton","doi":"10.1002/ajh.27722","DOIUrl":"https://doi.org/10.1002/ajh.27722","url":null,"abstract":"<div>\u0000<div>\u0000<p><i>The timing of your decision is just in important as the decision you make</i>.</p> John C. Maxwell </div>\u0000</div>\u0000<p>The management of CML has evolved over the last quarter century or so, transforming this once fatal disease into one that is curable or at least controllable [<span>1</span>]. With the routine use of tyrosine kinase inhibitor (TKI) therapy, life expectancy is near normal compared to age-matched controls [<span>2</span>] although quality of life (QOL) may not be normal [<span>3, 4</span>]. Survival has not really improved beyond what was seen with the first generation of these drugs, but newer drugs have helped with issues of resistance and intolerance, making the need to use more aggressive options a much less frequent event.</p>\u0000<p>In the pre-TKI era, the only option for possible “cure” with the rare exception of interferon therapy, was an allogeneic bone marrow, now stem cell transplant [<span>5, 6</span>]. This, however, was a reality if a patient was young, usually under the age of 40 years, had a matched sibling, had reasonable health, was near a transplant program, and had reimbursement for this costly procedure. In the western world, where the median age of CML diagnosis is in the mid-60s, or the developing world, where the procedure was not technically available or reimbursed, it can be seen that the majority of patients were left with palliative management only. If this was all that was available, major developments in allografting have occurred, which have changed the landscape. Patients can now be transplanted into their eighth decade, co-morbidities can often be managed, reduced intensity conditioning is less acutely toxic, better graft-versus-host disease (GVHD) prophylaxis and therapy are available, costs are down, and more centers are available. But most significantly, with the availability of a larger donor pool—alternate related donors, unrelated donor registries, and haploidentical donors—allografting could be available to more people [<span>7, 8</span>]. The converse of this is…so what. With the appearance of imatinib and subsequent TKIs, the need for allografting almost disappeared, and along with it, the memory that allografting still exists and may be appropriate in a small number of cases.</p>\u0000<p>In parallel, advancements in TKI development have yielded newer drugs that in many cases can deal with resistance or intolerance of earlier drugs and for some patients with co-morbidities, may be safer and without the problem of adverse events or side effects [<span>9, 10</span>]. This has reduced but not eliminated the need to consider an allograft. On the other hand, more frequent chronic problems can sometimes be replaced with more serious issues or, in cases of the newest drugs, unknown long-term issues, if any. Risk/benefit ratios become important, and the use of a potentially “riskier” drug can be justified with more advanced CML, after careful thought, discussion, and consent.</p>\u0000<p>This comm","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"35 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144097362","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":"Routine Screening of Strongyloides Among Patients With Lymphoma and Efficacy of Targeted Prophylaxis.","authors":"Michele D Stanchina,Camila Sacher,Yamila Melendez,Pamela Dudkiewicz,Jose F Camargo,Izidore S Lossos","doi":"10.1002/ajh.27719","DOIUrl":"https://doi.org/10.1002/ajh.27719","url":null,"abstract":"A total of 1372 lymphoma patients (980 newly diagnosed and 392 seen in cellular therapy clinic) were screened for Strongyloides serology, detecting IgG in 80 (6%) patients. Only one out of 80 (1.25%) patients diagnosed and treated with ivermectin for Strongyloides developed disseminated strongyloidiasis following chemotherapy treatments, supporting routine screening and prophylaxis in lymphoma patients.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"132 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144087774","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":"Classical and Late-Onset SOS/VOD After Allogeneic HSCT: A Japanese Transplant Registry Analysis.","authors":"Kyoko Masuda,Keisuke Kataoka,Masatoshi Sakurai,Yuho Najima,Naonori Harada,Shoko Ukita,Naoyuki Uchida,Noriko Doki,Takahiro Fukuda,Masatsugu Tanaka,Hiroyuki Ohigashi,Jun Ishikawa,Satoshi Yoshihara,Masashi Sawa,Shuichi Ota,Yoshinobu Kanda,Tetsuya Nishida,Makoto Onizuka,Yoshiko Atsuta,Hideki Nakasone,Kimikazu Yakushijin","doi":"10.1002/ajh.27715","DOIUrl":"https://doi.org/10.1002/ajh.27715","url":null,"abstract":"Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a lethal complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT). According to the 2016 European Society for Blood and Marrow Transplantation criteria, SOS/VOD is classified into classical SOS/VOD and late-onset SOS/VOD, but their similarities and differences remain unclear. Here we retrospectively investigated the incidence, risk factors, and impact on transplant outcomes of classical and late-onset SOS/VOD in 16 518 allo-HSCT recipients using the Japanese nationwide registry data. The cumulative incidences of classical and late-onset SOS/VOD were 2.5% and 2.2%, with a median onset of 13 and 42 days after transplantation, respectively. Both patients with classical (hazard ratio [HR], 3.45; 95% CI, 3.07-3.87) and late-onset (HR, 3.98; 95% CI, 3.51-4.51) SOS/VOD had a significantly worse overall survival compared with those without. The risk factors for classical and late-onset SOS/VOD are different. Hepatic comorbidities, high-risk diseases, use of melphalan (MEL), and myeloablative conditioning are associated with both types of SOS/VOD. Whereas poor performance status, a prior history of transplantation, and positive hepatitis C virus are associated with only classical SOS/VOD, allo-HSCT from cord blood or related human leukocyte antigen-haploidentical donors, use of total body irradiation and busulfan (BU), and tacrolimus-based graft-versus-host disease prophylaxis are associated with only late-onset SOS/VOD. In particular, the incidence of late-onset SOS/VOD is much higher in patients receiving both BU- and MEL-containing conditioning regimens. These findings suggest that different monitoring and treatment approaches are necessary for allo-HSCT recipients at high risk for classical and late-onset SOS/VOD.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"9 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144087745","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":"Cystatin C as Biomarker for the Evaluation of Renal Outcome in AL Amyloidosis.","authors":"Foteini Theodorakakou,Despina Fotiou,Filia Apostolakou,Ioannis Papassotiriou,Vasiliki Spiliopoulou,Ioannis Ntanasis-Stathopoulos,Panagiotis Malandrakis,Magdalini Migkou,Nikolaos Kanellias,Evangelos Eleutherakis-Papaiakovou,Erasmia Psimenou,Asimina Papanikolaou,Charikleia Gakiopoulou,Smaragdi Marinaki,Stavroula Giannouli,Maria Gavriatopoulou,Evangelos Terpos,Meletios-Athanasios Dimopoulos,Efstathios Kastritis","doi":"10.1002/ajh.27716","DOIUrl":"https://doi.org/10.1002/ajh.27716","url":null,"abstract":"Cystatin C (CysC) has emerged as a novel and potentially more reliable biomarker for the estimation of glomerular filtration in the general population in patients with various conditions. In AL amyloidosis, the current renal staging system and renal response criteria are based on proteinuria and creatinine-based eGFR. We explored the prognostic role of CysC and of estimation of eGFR based on CysC-based equations in a cohort of 195 patients with newly diagnosed AL amyloidosis with renal involvement. Baseline CysC level was strongly and independently associated with progression to dialysis, and CysC levels ≥ 1.9 mg/L can be used in combination with the current renal staging system to identify patients with different risk of progression to dialysis among renal stages 2 and 3. eGFR based on CysC performed at least similarly to eGFR based on creatinine alone (by CKD-EPI race free formula) and the cutoff of 30 mL/min/1.73 m2 could better predict progression to dialysis at 2 years. At 6 months landmark, an increase in CysC by ≥ 1 mg/L was associated with higher risk of progression to dialysis (HR: 19.8, 95% CI 6.5-60.5, p < 0.001); a reduction of CysC based eGFR ≥ 30% was also associated with poor renal outcome, with a prognostic performance similar to current renal progression criteria. In conclusion, CysC provides prognostic information regarding the renal outcomes in patients with AL amyloidosis independently of the established biomarkers, but requires further validation.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"16 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144087766","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":"Adult Acute Lymphoblastic Leukemia: 2025 Update on Diagnosis, Therapy, and Monitoring.","authors":"Hagop Kantarjian,Elias Jabbour","doi":"10.1002/ajh.27708","DOIUrl":"https://doi.org/10.1002/ajh.27708","url":null,"abstract":"DISEASE OVERVIEWAcute lymphoblastic leukemia (ALL) is a disease of lymphoid progenitor cells arising in the bone marrow and extramedullary sites. While it is the most common pediatric cancer, ALL is a rare disease overall, with approximately 6500 new cases diagnosed in the United States, in 2024. Current treatment relies on multiagent chemotherapy administered over 2-3 years, resulting in long-term survival in 80%-90% in pediatric patients compared to 40%-50% in adult patients, depending upon patient- and disease-specific characteristics.PHILADELPHIA CHROMOSOME-POSITIVE B-CELL ALLHistorically considered a poor risk ALL subtype, the treatment and outcome of Philadelphia chromosome (Ph)-positive B-cell ALL were drastically changed with the advent of the BCR::ABL1 tyrosine kinase inhibitors (TKIs). The combination of a TKI with a backbone of multiagent chemotherapy, or more recently blinatumomab, is the mainstay of therapy, resulting in 5-year survival rates of 80+%. Achieving a complete molecular remission, particularly by next generation sequencing, is an important prognostic indicator, which may identify patients who may avoid allogeneic stem cell transplantation (SCT).PHILADELPHIA CHROMOSOME-NEGATIVE B-CELL ALLThe treatment approach for patients with Ph-negative B-cell ALL was historically composed of a chemotherapy backbone (either pediatric-inspired, or Hyper-CVAD based). Novel agents including inotuzumab ozogamicin and blinatumomab are being incorporated into these regimens to improve the rates of measurable residual disease negativity and long-term outcomes. While differences in long-term survival rates differ between age groups, such as adolescents and young adults compared to older adults (≥ 60 years), with these immunotherapy-chemotherapy regimens, the 4-year survival rates have improved to 80%-85% among patients who are able to receive these treatments. Elderly patients represent a difficult population to treat due to poor chemotherapy tolerance, high-risk disease features, and increased risk of developing therapy-related myeloid neoplasms. The use of inotuzumab ozogamicin and blinatumomab in lieu of intensive chemotherapy in this population has improved safety and efficacy in patients ≥ 60 years old. Clinical trials incorporating chimeric antigen receptor (CAR) T-cell therapy into treatment for older patients are in progress.T-CELL ALLCombination chemotherapy regimens incorporating pegylated asparaginase and nelarabine are the standard for patients with T-cell ALL. Early T-cell precursor (ETP) ALL is a high-risk subgroup for which allogeneic SCT should be considered. Inclusion of the BCL-2 inhibitor venetoclax into treatment for patients with ETP-ALL may be beneficial and is currently being investigated.SALVAGE THERAPYSeveral therapies are approved as single agents in the salvage setting. However, the best outcomes are obtained with combination therapy including chemo- and immuno- therapies followed by CAR T-cell consolidation and allog","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"69 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144065797","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":"Clinical Factors Associated With Catheter-Related VTE in Patients Undergoing Hematopoietic Cell Transplantation: A Multi-Center Study.","authors":"Navel Gopal Subramanian,Danielle Guffey,Jonathan Avery,David Garcia,Ryan Basom,Stephanie J Lee,Katherine Klein,Partow Kebriaei,Gabriela Rondon,Elizabeth Shpall,Shida Jin,Elliana Young,Cristhiam Mauricio Rojas Hernandez,Ang Li","doi":"10.1002/ajh.27720","DOIUrl":"https://doi.org/10.1002/ajh.27720","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"17 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144065796","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":"The Mutational Landscape in Polycythemia Vera: Phenotype, Genotype, and Prognostic Correlates.","authors":"Masooma S Rana,Moazah Iftikhar,Yamna Jadoon,Maymona Abdelmagid,David S Viswanatha,Rong He,Kaaren K Reichard,Animesh D Pardanani,Naseema Gangat,Ayalew Tefferi","doi":"10.1002/ajh.27717","DOIUrl":"https://doi.org/10.1002/ajh.27717","url":null,"abstract":"Polycythemia vera (PV) is invariably associated with a JAK2 mutation, with over 50% of patients harboring additional non-JAK2 mutations. In the current study, 319 patients with PV underwent NGS at diagnosis or in chronic phase PV (Group A: N = 270, 85%) or at the time of fibrotic (Group B; N = 37, 12%) or leukemic (Group C; N = 12, 4%) transformation. Mutational frequencies involving TP53/SRSF2/IDH1/U2AF1 were significantly (p < 0.05) different between patients in the mutually exclusive Groups A (2%/4%/2%/0.4%), B (8%/0%/0%/5%), and C (50%/25%/17%/8%). Analyses on phenotype/genotype associations and prognostic impact on overall (OS), leukemia-free (LFS), and myelofibrosis-free (MFFS) survival were limited to Group A patients. ASXL1MUT was associated with younger age (p < 0.01), SRSF2MUT with older age and leukocytosis (p < 0.01), and TP53MUT with leukocytosis (p < 0.01). Mutation co-segregation was apparent between ASXL1 and IDH2 (p < 0.01) or SRSF2 (p < 0.01), SRSF2 and IDH2 (p < 0.01), and TP53 and NRAS (p = 0.01). Multivariable analysis identified SRSF2MUT (p < 0.01; HR, 4.2, 1.9-9.5), IDH2MUT (p = 0.01; HR, 5.3, 1.8-15.3), ASXL1MUT (p = 0.04; HR, 2.0, 1.1-3.7), leukocyte count ≥ 15 × 109/L (p < 0.01; HR 2.0, 1.3-3.1), and advanced age (p < 0.01) as risk factors for OS. Median OS in the presence (N = 235; 87%) or absence (N = 35; 13%) of any adverse mutation (i.e., SRSF2MUT, ASXL1MUT, or IDH2MUT) was 8.8 versus 17.8 years (p = 0.01; HR 1.8, 1.1-2.9). In addition, ASXL1MUT (p = 0.02; HR, 1.6-24.9), SRSF2MUT (p = 0.06; HR, 11.9, 1.1-126.2), and advanced age (p = 0.04) were associated with inferior LFS, and SRSF2MUT (p < 0.01; HR, 24.0, 5.5-103.8) and abnormal karyotype (p < 0.01; HR 3.8, 1.6-8.9) with inferior MFFS. The number of non-JAK2 mutations was significant in predicting outcome in univariate but not multivariable analysis. The observations from the current study highlight the prognostic significance of non-JAK2 mutations in PV and the prospect of their inclusion in future prognostic models.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"129 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143992001","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}