American Journal of Hematology最新文献

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ALK+ Large B-Cell Lymphoma With Novel ZNF296::ALK: A Morphologic Mimic of ALK+ Anaplastic Large Cell Lymphoma. ALK+大b细胞淋巴瘤伴新型ZNF296::ALK: ALK+间变性大细胞淋巴瘤的形态学模拟物。
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-27 DOI: 10.1002/ajh.27727
Wei J Wang,Pei Lin,John M Stewart,Guilin Tang,Shaoying Li,Sanam Loghavi,L Jeffrey Medeiros,Jie Xu
{"title":"ALK+ Large B-Cell Lymphoma With Novel ZNF296::ALK: A Morphologic Mimic of ALK+ Anaplastic Large Cell Lymphoma.","authors":"Wei J Wang,Pei Lin,John M Stewart,Guilin Tang,Shaoying Li,Sanam Loghavi,L Jeffrey Medeiros,Jie Xu","doi":"10.1002/ajh.27727","DOIUrl":"https://doi.org/10.1002/ajh.27727","url":null,"abstract":"","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"12 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144146166","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}
引用次数: 0
Impact of Conditioning Intensity on Clinical Outcomes of Second Allogeneic Hematopoietic Cell Transplantation for Relapse After First Transplantation 调节强度对第一次移植后复发的第二次异体造血细胞移植临床结果的影响。
IF 10.1 1区 医学
American Journal of Hematology Pub Date : 2025-05-23 DOI: 10.1002/ajh.27709
Kazuki Yoshimura, Hideki Nakasone, Masaharu Tamaki, Hiroki Hosoi, Kazuaki Kameda, Naoyuki Uchida, Noriko Doki, Takahiro Fukuda, Satoshi Yoshihara, Yasuo Mori, Hirohisa Nakamae, Masatsugu Tanaka, Yuta Katayama, Tetsuya Eto, Yuta Hasegawa, Shuichi Ota, Satoshi Takahashi, Makoto Yoshimitsu, Fumihiko Ishimaru, Junya Kanda, Yoshiko Atsuta, Kimikazu Yakushijin
{"title":"Impact of Conditioning Intensity on Clinical Outcomes of Second Allogeneic Hematopoietic Cell Transplantation for Relapse After First Transplantation","authors":"Kazuki Yoshimura,&nbsp;Hideki Nakasone,&nbsp;Masaharu Tamaki,&nbsp;Hiroki Hosoi,&nbsp;Kazuaki Kameda,&nbsp;Naoyuki Uchida,&nbsp;Noriko Doki,&nbsp;Takahiro Fukuda,&nbsp;Satoshi Yoshihara,&nbsp;Yasuo Mori,&nbsp;Hirohisa Nakamae,&nbsp;Masatsugu Tanaka,&nbsp;Yuta Katayama,&nbsp;Tetsuya Eto,&nbsp;Yuta Hasegawa,&nbsp;Shuichi Ota,&nbsp;Satoshi Takahashi,&nbsp;Makoto Yoshimitsu,&nbsp;Fumihiko Ishimaru,&nbsp;Junya Kanda,&nbsp;Yoshiko Atsuta,&nbsp;Kimikazu Yakushijin","doi":"10.1002/ajh.27709","DOIUrl":"10.1002/ajh.27709","url":null,"abstract":"<div>\u0000 \u0000 <p>Although second allogeneic hematopoietic cell transplantation HCT (HCT2) is a potentially curative treatment for patients relapsing after their first HCT (HCT1), it is associated with higher non-relapse mortality (NRM) compared with HCT1. Furthermore, while reduced-intensity conditioning (RIC) in HCT2 might decrease NRM, there is no consensus on which patients may benefit from RIC. We retrospectively analyzed 2478 patients who underwent HCT2 for relapse of hematologic malignancies after HCT1. In a multivariate analysis, older recipient age, short duration between HCT1 and HCT2, RIC in HCT1, HCT-CI ≥ 2, and ECOG PS ≥ 2 were associated with an increased risk of NRM. RIC in HCT2 was associated with better NRM compared to myeloablative conditioning (MAC) (hazard ratio [HR] 0.83, 95% confidence interval [CI]: 0.72–0.97; <i>p</i> = 0.018), but was not significantly associated with overall survival (OS) (HR 0.91, 95% CI: 0.82–1.01; <i>p</i> = 0.075). We observed a significant interaction for NRM between extensive cGVHD in HCT1 and the conditioning intensity of HCT2 (interaction <i>p</i> &lt; 0.001), meaning that the benefit of RIC in HCT2 was seen in patients with extensive cGVHD in HCT1, but not in those without cGVHD. RIC in HCT2 was also associated with superior OS in patients with extensive cGVHD in HCT1 (HR 0.68, 95% CI: 0.49–0.93; <i>p</i> = 0.02), with significant interaction between the conditioning intensity and the prior history of extensive cGVHD (interaction <i>p</i> = 0.01). This study suggests that RIC in HCT2 reduces NRM for HCT2 and improves OS, especially in patients with a history of extensive cGVHD.</p>\u0000 </div>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"100 7","pages":"1185-1195"},"PeriodicalIF":10.1,"publicationDate":"2025-05-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144122226","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}
引用次数: 0
A Phase 2 Trial of CPX-351 Combined With Venetoclax in Relapsed or Refractory Acute Myeloid Leukemia. CPX-351联合Venetoclax治疗复发或难治性急性髓系白血病的2期临床试验
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-22 DOI: 10.1002/ajh.27723
Tapan M Kadia,Wei-Ying Jen,Alex Bataller,Alexandre Bazinet,Gautam Borthakur,Elias Jabbour,Wei Qiao,Nicholas J Short,Koichi Takahashi,Ghayas C Issa,Courtney D DiNardo,Guillermo Montalban-Bravo,Naveen Pemmaraju,Andrew Tran,Vanthana Bharathi,Sanam Loghavi,Amin M Alousi,Uday Popat,Naval G Daver,Farhad Ravandi,Hagop M Kantarjian
{"title":"A Phase 2 Trial of CPX-351 Combined With Venetoclax in Relapsed or Refractory Acute Myeloid Leukemia.","authors":"Tapan M Kadia,Wei-Ying Jen,Alex Bataller,Alexandre Bazinet,Gautam Borthakur,Elias Jabbour,Wei Qiao,Nicholas J Short,Koichi Takahashi,Ghayas C Issa,Courtney D DiNardo,Guillermo Montalban-Bravo,Naveen Pemmaraju,Andrew Tran,Vanthana Bharathi,Sanam Loghavi,Amin M Alousi,Uday Popat,Naval G Daver,Farhad Ravandi,Hagop M Kantarjian","doi":"10.1002/ajh.27723","DOIUrl":"https://doi.org/10.1002/ajh.27723","url":null,"abstract":"Outcomes in patients with relapsed/refractory (RR) AML are poor. We sought to investigate if CPX-531 in combination with venetoclax (CPX + VEN) was tolerable and effective in RR AML. This was a single institution phase 1b/2 trial of CPX + VEN. Patients aged ≥ 18 years with RR AML who were fit for intensive chemotherapy were eligible. Prior venetoclax exposure was allowed. The phase 1b portion followed a 3 + 3 design to identify the recommended phase 2 dose (RP2D) for the expansion cohort. At the starting dose level of -1, prolonged myelosuppression was observed, leading to dose level -2 (CPX-351 dosed at daunorubicin 44 mg/m2 on days 1,3, and 5 and venetoclax 300 mg days 2-8) being chosen as the RP2D. Thirty three patients with a median age of 58 years (range, 26-72) were treated. Patients were heavily pretreated, with 58% with prior venetoclax exposure, 44% in the second salvage or later, and 30% with prior stem cell transplant (SCT). Adverse cytogenetics were present in 51% of patients, with myelodysplasia-related mutations in 64%, and TP53mut in 21%. The overall response rate (ORR) was 46% (95% CI, 30-62), with a composite CR rate (CRc) of 39% (95% CI, 25-56). Patients in first salvage with wildtype TP53 had a CRc rate of 70% (95% CI, 40-89), with undetectable MRD in 71% (95% CI, 36-92) and a 2-year OS of 49% (95% CI, 23-100). Eleven (73%) responding patients underwent SCT. The 30-day mortality was 9%, with a 60-day mortality of 21%. The most common adverse events were related to myelosuppression. CPX + VEN has activity in RR AML, particularly when used in first salvage and in patients who do not harbor TP53 mutations. ClinicalTrials.gov Identifier: NCT03629171.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"31 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144114185","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}
引用次数: 0
PET/CT in the Staging and Treatment Response Assessment of Patients With Extranodal Marginal Zone Lymphoma. PET/CT在结外边缘区淋巴瘤患者分期及治疗反应评估中的应用。
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-21 DOI: 10.1002/ajh.27712
Juan Pablo Alderuccio,Russ A Kuker,Eduardo Edelman Saul,Michele D Stanchina,Mark K Polar,Jennifer Chapman,Wei Zhao,Rafael Hennemann Sassi,Craig H Moskowitz,Isildinha M Reis,Izidore S Lossos
{"title":"PET/CT in the Staging and Treatment Response Assessment of Patients With Extranodal Marginal Zone Lymphoma.","authors":"Juan Pablo Alderuccio,Russ A Kuker,Eduardo Edelman Saul,Michele D Stanchina,Mark K Polar,Jennifer Chapman,Wei Zhao,Rafael Hennemann Sassi,Craig H Moskowitz,Isildinha M Reis,Izidore S Lossos","doi":"10.1002/ajh.27712","DOIUrl":"https://doi.org/10.1002/ajh.27712","url":null,"abstract":"18Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) is the standard imaging modality in lymphoma. The 2014 Lugano classification considers extranodal marginal zone lymphoma (EMZL) a non-FDG-avid disease, recommending contrast-enhanced CT. We reassessed the utility of PET/CT for staging workup and response assessment of EMZL. We reviewed staging and response PET/CT of 190 EMZL sites from 152 patients. Each location was counted independently for patients with > 1 extranodal site. Although not standard, we considered FDG-avid disease if SUVmax was ≥ 2, and calculated ratios between lymphoma SUVmax and mediastinal blood pool (BP index) and liver background (liver index). FDG avidity was detected in 151 (79.5%) out of 190 extranodal sites (in 117 [76.7%] out of 152 patients), with a median SUVmax of 4.5 (IQR 2.5-6.9, range 0-26.8). Locations showing FDG avidity in > 90% of extranodal sites included salivary gland, bone, lung, soft tissue, ocular adnexa, and airways. Skin was commonly non-FDG avid (93.8%). Among 22 patients with > 1 extranodal location, there was concordant FDG avidity in all sites in 18 (81.8%) patients. Considering measurable extranodal disease size > 0.5 cm, we observed significant Pearson correlation coefficients (r) between lymphoma size and SUVmax (r = 0.20, p = 0.019, n = 142), BP index (r = 0.34, p < 0.001, n = 124), and liver index (r = 0.36, p < 0.001, n = 124). We also observed improved precision in response to treatment assessment in FDG-avid EMZL tumors. This study demonstrates that EMZL is commonly an FDG-avid disease, suggesting that PET/CT should be routinely used in the staging and response assessment workup of patients with EMZL.","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"40 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144103685","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}
引用次数: 0
Correction to “Efficacy and Safety of Early-Start Deferiprone in Infants and Young Children With Transfusion-Dependent Beta Thalassemia: Evidence for Iron Shuttling to Transferrin in a Randomized, Double-Blind, Placebo-Controlled, Clinical Trial (START)” 对“早启动去铁素治疗输血依赖性地中海贫血婴幼儿的疗效和安全性:一项随机、双盲、安慰剂对照的临床试验(START)中铁转运到转铁蛋白的证据”的修正
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-21 DOI: 10.1002/ajh.27703
{"title":"Correction to “Efficacy and Safety of Early-Start Deferiprone in Infants and Young Children With Transfusion-Dependent Beta Thalassemia: Evidence for Iron Shuttling to Transferrin in a Randomized, Double-Blind, Placebo-Controlled, Clinical Trial (START)”","authors":"","doi":"10.1002/ajh.27703","DOIUrl":"https://doi.org/10.1002/ajh.27703","url":null,"abstract":"<p>M. S. Elalfy, M. Hamdy, A. Adly, et al., “Efficacy and Safety of Early-Start Deferiprone in Infants and Young Children With Transfusion-Dependent Beta Thalassemia: Evidence for Iron Shuttling to Transferrin in a Randomized, Double-Blind, Placebo-Controlled, Clinical Trial (START),” <i>American Journal of Hematology</i> 98, no. 9 (2023): 1415–1424, https://doi.org/10.1002/ajh.27010.</p>\u0000<p>In the acknowledgements and in the Supplemental Figure 1, the paper omits to mention the contribution made by the study sites in Indonesia and the Pediatric Hospital of Cairo. As these sites are not represented in the author's masthead, we would like to remedy this under the provision given in your correction reasons: “Acknowledgements of funder support or non-author contributors have not been included; or declarations related to ethics approval or conflicts of interest need to be added.”</p>\u0000<p>To support this, we have pasted the desired acknowledgement section below and appended the proposed Supplemental Figure 1 (the addition there being the addition of the Study Site table at the bottom).</p>\u0000<p>This correction has been discussed and approved by all authors, contributors and sponsors.</p>\u0000<p>PROPOSED NEW ACKNOWLEDGEMENTS</p>\u0000<p>The authors thank the patients and their families for participation in this study. The authors would also like to acknowledge the contributions of Professor Amal Beshlawy and her team at Pediatric Hospital of Cairo University (Cairo, Egypt) and Prof Pustika Amalia Wahidiyat and her team at Rumah Sakit Umum Pusat National Dr. Cipto Mangunkusumo (Jakarta, Indonesia), who were investigators and study sites. The study was sponsored by Chiesi Global Rare Diseases (formerly ApoPharma Inc.), Boston, MA, USA. Medical writing support was provided by Keith M. Olson, PhD, and Emily K. LaVigne, PhD, of Oxford PharmaGenesis Inc., Newtown, PA, USA, and funded by Chiesi USA Inc.</p>\u0000<div>We apologize for this error. <figure>\u0000<div><picture>\u0000<source media=\"(min-width: 1650px)\" srcset=\"/cms/asset/a39bd6df-d60b-436c-8642-54684397ca23/ajh27703-gra-0001-m.jpg\"/><img alt=\"image\" data-lg-src=\"/cms/asset/a39bd6df-d60b-436c-8642-54684397ca23/ajh27703-gra-0001-m.jpg\" loading=\"lazy\" src=\"/cms/asset/6ee718b8-7722-46d2-9a7d-785ba802c6c8/ajh27703-gra-0001-m.png\" title=\"image\"/></picture><p></p>\u0000</div>\u0000</figure>\u0000</div>","PeriodicalId":7724,"journal":{"name":"American Journal of Hematology","volume":"18 1","pages":""},"PeriodicalIF":12.8,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144104172","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}
引用次数: 0
Corrigendum to "Pyruvate Kinase Function Correlates With Red Blood Cell Properties and Clinical Manifestations in Sickle Cell Disease". 《镰状细胞病中与红细胞特性和临床表现相关的丙酮酸激酶功能》的勘误。
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-21 DOI: 10.1002/ajh.27713
{"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}
引用次数: 0
Efficacy and Safety of the Bruton's Tyrosine Kinase Inhibitor Zanubrutinib in Immune Thrombocytopenia 布鲁顿酪氨酸激酶抑制剂扎鲁替尼治疗免疫性血小板减少症的疗效和安全性
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-20 DOI: 10.1002/ajh.27718
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
{"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 × 10<sup>9</sup>/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) × 10<sup>9</sup>/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}
引用次数: 0
Mixed Autoimmune Hemolytic Anemia: A Systematic Review of Epidemiology, Clinical Characteristics, Therapies, and Outcomes 混合自身免疫性溶血性贫血:流行病学、临床特征、治疗和结果的系统综述
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-20 DOI: 10.1002/ajh.27721
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
{"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}
引用次数: 0
Watching the Barn Door Open—Timing CML Stem Cell Allografts 观察谷仓门打开时间CML干细胞同种异体移植
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-20 DOI: 10.1002/ajh.27722
Jeffrey H. Lipton
{"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":"&lt;div&gt;\u0000&lt;div&gt;\u0000&lt;p&gt;&lt;i&gt;The timing of your decision is just in important as the decision you make&lt;/i&gt;.&lt;/p&gt; John C. Maxwell &lt;/div&gt;\u0000&lt;/div&gt;\u0000&lt;p&gt;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 [&lt;span&gt;1&lt;/span&gt;]. With the routine use of tyrosine kinase inhibitor (TKI) therapy, life expectancy is near normal compared to age-matched controls [&lt;span&gt;2&lt;/span&gt;] although quality of life (QOL) may not be normal [&lt;span&gt;3, 4&lt;/span&gt;]. 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.&lt;/p&gt;\u0000&lt;p&gt;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 [&lt;span&gt;5, 6&lt;/span&gt;]. 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 [&lt;span&gt;7, 8&lt;/span&gt;]. 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.&lt;/p&gt;\u0000&lt;p&gt;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 [&lt;span&gt;9, 10&lt;/span&gt;]. 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.&lt;/p&gt;\u0000&lt;p&gt;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}
引用次数: 0
Routine Screening of Strongyloides Among Patients With Lymphoma and Efficacy of Targeted Prophylaxis. 淋巴瘤患者类圆线虫的常规筛查及针对性预防的效果。
IF 12.8 1区 医学
American Journal of Hematology Pub Date : 2025-05-19 DOI: 10.1002/ajh.27719
Michele D Stanchina,Camila Sacher,Yamila Melendez,Pamela Dudkiewicz,Jose F Camargo,Izidore S Lossos
{"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}
引用次数: 0
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