Acta Haematologica最新文献_第2页

Acute Myeloid Leukemia Treatment in the Elderly: A Comprehensive Review of the Present and Future. 老年人急性髓性白血病的治疗：现状与未来的全面回顾。

IF 1.7 4区医学

Acta Haematologica Pub Date : 2023-01-01 Epub Date: 2023-07-17 DOI: 10.1159/000531628

Jun H Choi, Mihir Shukla, Maher Abdul-Hay

{"title":"Acute Myeloid Leukemia Treatment in the Elderly: A Comprehensive Review of the Present and Future.","authors":"Jun H Choi, Mihir Shukla, Maher Abdul-Hay","doi":"10.1159/000531628","DOIUrl":"10.1159/000531628","url":null,"abstract":"Background: Acute myeloid leukemia (AML) is a disease of the hematopoietic system that remains a therapeutic challenge despite advances in our understanding of the underlying cancer biology in the past decade. It is also an affliction of the elderly that predominantly affects patients over 60 years of age. Standard therapy involves intensive chemotherapy that is often difficult to tolerate in older populations. Fortunately, recent developments in molecular targeting have shown promising results in treating leukemia, paving the way for novel treatment strategies that are easier to tolerate.Summary: Venetoclax, a BCL-2 inhibitor, when combined with a hypomethylating agent, has proven to be a highly effective and well-tolerated drug and established itself as a new standard for treating AML in patients who are unfit for standard intensive therapy. Other targeted therapies include clinically proven and FDA-approved agents, such as IDH1/2 inhibitors, FLT3 inhibitors, and Gemtuzumab, as well as newer and more experimental drugs such as magrolimab, PI-kinase inhibitors, and T-cell engaging therapy. Some of the novel agents such as magrolimab and menin inhibitors are particularly promising, providing therapeutic options to a wider population of patients than ever before. Determining who will benefit from intense or novel low-intense therapy remains a challenge, and it requires careful assessment of individual patient's fitness and disease characteristics.Key messages: This article reviews past and current treatment strategies that harness various mechanisms of leukemia-targeting agents and introduces novel therapies on the horizon aimed at exploring therapeutic options for the elderly and unfit patient population. It also provides a strategy to select the best available therapy for elderly patients with both newly diagnosed and relapsed/refractory AML.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"431-457"},"PeriodicalIF":1.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10203483","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

Prediction of Splenectomy Outcomes in Relapsed or Refractory Immune Thrombocytopenia. 复发或难治性免疫性血小板减少症患者脾切除术预后的预测。

IF 2.4 4区医学

Acta Haematologica Pub Date : 2023-01-01 DOI: 10.1159/000530678

Philip Murphy

引用次数: 0

The Role of Follicular Regulatory T Cells/Follicular Helper T Cells in Primary Immune Thrombocytopenia. 滤泡调节性T细胞/滤泡辅助性T细胞在原发性免疫性血小板减少症中的作用

IF 2.4 4区医学

Acta Haematologica Pub Date : 2023-01-01 DOI: 10.1159/000529963

Mingling Sun, Xiujuan Wang, Lei Wang, Xinyou Wang, Wenxia Fan, Ying Liu, Qinzhi Li, Ning Zhang, Xinhong Guo

{"title":"The Role of Follicular Regulatory T Cells/Follicular Helper T Cells in Primary Immune Thrombocytopenia.","authors":"Mingling Sun, Xiujuan Wang, Lei Wang, Xinyou Wang, Wenxia Fan, Ying Liu, Qinzhi Li, Ning Zhang, Xinhong Guo","doi":"10.1159/000529963","DOIUrl":"https://doi.org/10.1159/000529963","url":null,"abstract":"Introduction: Immune thrombocytopenia (ITP) is an autoimmune disease characterized by thrombocytopenia. Herein, we sought to identify potential immune-related therapeutic targets in ITP.Methods: The differentially expressed genes (DEGs) between ITP patients and controls in GSE43177 and PRJNA299534 were analyzed. The intersections of the two DEG groups were screened as common genes, and enrichment analysis was performed. Additionally, differential analysis of immune cell levels between ITP and controls was performed. Changes in the proportions of T follicular helper (Tfh) and follicular regulatory T (Tfr) cells in peripheral blood samples from ITP patients, ITP patients responding to therapy, and healthy controls were identified. The expression changes in B-cell lymphoma (Bcl)-6 and interleukin (IL)-21 were further evaluated.Results: A total of 76 common genes were identified, and enrichment analysis found that these genes were mainly associated with neutrophil-mediated immunity, the MAPK signaling pathway, and the FOXO signaling pathway. Furthermore, we found different levels of Tfh cells in patients with ITP and controls. The level of Tfh cells in the peripheral blood is significantly increased in ITP patients and declines after responding to therapy. The Tfr/Tfh ratio was reduced in ITP patients and increased after responding to therapy. IL-21 and Bcl-6 were more highly expressed in ITP patients than in controls.Conclusion: We identified abnormally expressed genes in ITP related to immune-related biological functions. We further identified the changes in Tfh and Tfr cells during ITP treatment. This provides a rationale for immunotherapy in ITP patients.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":"146 4","pages":"267-276"},"PeriodicalIF":2.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9983998","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

Prognostic Significance of CD20 Expression in Children with Philadelphia Chromosome-Negative B-Cell Precursor Acute Lymphoblastic Leukemia. CD20在费城染色体阴性B细胞前体急性淋巴细胞白血病患儿中表达的预后意义。

IF 2.4 4区医学

Acta Haematologica Pub Date : 2023-01-01 Epub Date: 2023-05-07 DOI: 10.1159/000530849

Yu Wang, Yu-Juan Xue, Yue-Ping Jia, Ying-Xi Zuo, Ai-Dong Lu, Le-Ping Zhang

{"title":"Prognostic Significance of CD20 Expression in Children with Philadelphia Chromosome-Negative B-Cell Precursor Acute Lymphoblastic Leukemia.","authors":"Yu Wang, Yu-Juan Xue, Yue-Ping Jia, Ying-Xi Zuo, Ai-Dong Lu, Le-Ping Zhang","doi":"10.1159/000530849","DOIUrl":"10.1159/000530849","url":null,"abstract":"Introduction: The prognostic significance of CD20 in pediatric B-cell precursor acute lymphoblastic leukemia (BCP-ALL) remains unclear. Therefore, in this study, we evaluated the prognostic value of CD20 expression in leukemia blasts in pediatric BCP-ALL at our institute.Methods: Between 2005 and 2017, 796 children with newly diagnosed Philadelphia-negative BCP-ALL were enrolled consecutively; clinical characteristics and treatment outcomes were analyzed and compared between CD20-positive and CD20-negative groups.Results: CD20 positivity was observed in 22.7% of enrolled patients. The analysis of overall and event-free survival showed that white blood cell count ≥50 × 109/L, no ETV6-RUNX1, day 33 minimal residual disease (MRD) ≥0.1%, and week 12 MRD ≥0.01% were independent risk factors. Meanwhile, in the CD20-positive group, week 12 MRD ≥0.01% was the only factor associated with long-term survival. Moreover, subgroup analysis revealed that in patients with extramedullary involvement (p = 0.047), MRD ≥0.1% on day 33 (p = 0.032), or MRD ≥0.01% at week 12 (p = 0.004), CD20 expression led to a poorer outcome compared to those without CD20 expression.Conclusions: Pediatric BCP-ALL with CD20 expression had unique clinicopathological characteristics, and MRD remained the major prognostic factor. CD20 expression had no prognostic value in pediatric BCP-ALL.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"349-357"},"PeriodicalIF":2.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9754731","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

Impact of Geographical Distance from Quaternary Treatment Center on Clinical Trial Participation, Intensive Induction Chemotherapy, and Outcomes in Patients with Newly Diagnosed Acute Myeloid Leukemia. 与第四纪治疗中心的地理距离对新诊断急性髓细胞白血病患者临床试验参与、强化诱导化疗和结果的影响。

IF 2.4 4区医学

Acta Haematologica Pub Date : 2023-01-01 Epub Date: 2023-06-14 DOI: 10.1159/000531484

Samantha A Hershenfeld, Eshetu G Atenafu, Steven Chan, Vikas Gupta, Dawn Maze, Andre Schuh, Hassan Sibai, Karen Yee, Arron Schimmer

{"title":"Impact of Geographical Distance from Quaternary Treatment Center on Clinical Trial Participation, Intensive Induction Chemotherapy, and Outcomes in Patients with Newly Diagnosed Acute Myeloid Leukemia.","authors":"Samantha A Hershenfeld, Eshetu G Atenafu, Steven Chan, Vikas Gupta, Dawn Maze, Andre Schuh, Hassan Sibai, Karen Yee, Arron Schimmer","doi":"10.1159/000531484","DOIUrl":"10.1159/000531484","url":null,"abstract":"Introduction: Care for patients with acute myeloid leukemia (AML) is centralized in the Ontario single-payer public healthcare system, with intensive induction chemotherapy and clinical trials only offered at specialized cancer centers with large catchment areas.Methods: We therefore conducted a retrospective single-center review of all AML patients assessed at a large specialized cancer center in Ontario, Canada.Results: Between 2012 and 2017, 1,310 patients were assessed by our center for upfront AML therapy. The median distance was 33.1 km, with 29% of patients living more than 50 km away from the center. There was no significant difference in probability of intensive induction chemotherapy or clinical trial by distance from center, both in univariate and multivariable analysis adjusting for age, sex, cytogenetics and molecular testing, and performance status. There was no significant difference in overall survival by distance from center on univariate and multivariable analysis.Conclusion: In conclusion, geographic distance from treatment center does not appear to impact choice of upfront therapy, participation in clinical trials, or clinical outcomes in this study of newly diagnosed patients with AML treated in a single-payer environment.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"366-372"},"PeriodicalIF":2.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9632287","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

Erdheim-Chester Disease with BRAF V600E Mutation and a Concomitant Myeloid Malignancy Sharing NRAS and IDH2 Mutations. 伴有BRAF V600E突变和髓系恶性肿瘤共享NRAS和IDH2突变的埃尔德海姆-切斯特病

IF 2.4 4区医学

Acta Haematologica Pub Date : 2023-01-01 DOI: 10.1159/000528550

Nitya Prabhakaran, George Jour, Arjun Balar, Nicholas Ward

{"title":"Erdheim-Chester Disease with BRAF V600E Mutation and a Concomitant Myeloid Malignancy Sharing NRAS and IDH2 Mutations.","authors":"Nitya Prabhakaran, George Jour, Arjun Balar, Nicholas Ward","doi":"10.1159/000528550","DOIUrl":"https://doi.org/10.1159/000528550","url":null,"abstract":"Erdheim-Chester disease (ECD) is a rare clonal histiocytic process that is characterized by a foamy (xanthomatous) proliferation often associated with Touton giant cells. The diagnosis is often challenging and not exclusively a histologic diagnosis, as it requires correlation with unique clinical, radiographic, and recently described molecular findings. Activating mutations involving the MAPK pathway including BRAF, ARAF, N/KRAS, and MEK are recurrent in the disease. However, it is increasingly being described that mutations associated with clonal hematopoiesis are also found in bone marrow specimens of patients with ECD, as well as higher frequency of overt concomitant myeloid malignancy including acute myeloid leukemia, myeloproliferative neoplasms, myelodysplastic syndromes, and mixed myeloproliferative neoplasms/myelodysplastic syndromes. Herein, we report a unique case of a patient presenting with BRAFV600E-positive ECD with peripheral blood findings consistent with a concurrent myeloid malignancy featuring co-occurrence of NRAS and IDH2 mutations.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":"146 3","pages":"245-251"},"PeriodicalIF":2.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10273903/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9648333","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

Adverse Effects of COVID-19 Vaccination in Patients with Transfusion-Dependent Thalassemia: An Observational Study from a Tertiary Care Center in Punjab, India. 新冠肺炎疫苗接种对输血依赖性地中海贫血患者的不良影响：印度旁遮普省三级护理中心的一项观察研究。

IF 1.7 4区医学

Acta Haematologica Pub Date : 2023-01-01 Epub Date: 2023-06-16 DOI: 10.1159/000531448

Shruti Kakkar, Evani Jain, Anirudh Jain, Priyanka Dewan

{"title":"Adverse Effects of COVID-19 Vaccination in Patients with Transfusion-Dependent Thalassemia: An Observational Study from a Tertiary Care Center in Punjab, India.","authors":"Shruti Kakkar, Evani Jain, Anirudh Jain, Priyanka Dewan","doi":"10.1159/000531448","DOIUrl":"10.1159/000531448","url":null,"abstract":"Vaccination against SARS-CoV-2 is the most important weapon in the arsenal in the battle against COVID-19. There is concern about an increased risk of adverse effects in patients with transfusion-dependent thalassemia (TDT), which affects vaccine acceptance. A predesigned questionnaire was used to evaluate adverse effects (local/systemic within 90 days after vaccination) in participants >18 years of age with TDT. A total of 100 patients received 129 vaccine doses. The mean age of the patients was 24.3 ± 5.7 years, and M:F ratio of 1.6:1. Covishield (Serum Institute of India) was administered to 89% of the participants, and Covaxin (Bharat Biotech Limited) to 11% of the participants. Adverse effects were documented in 62% of the respondents and were more pronounced after the first dose (52%) compared to the second dose (9%). The most frequent adverse effects were pain at the injection site (43%) and fever (37%). All adverse effects were mild and none of the participants required hospitalization. There were no differences in adverse effects among different vaccines, in the presence or absence of comorbidities, blood groups, or ferritin levels. The SARS-CoV-2 vaccine appears to be safe for patients with TDT.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":" ","pages":"391-396"},"PeriodicalIF":1.7,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11251649/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9649200","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

Relapses of Immune Thrombocytopenia after the Second and Booster Doses of BNT162b2 Vaccine. 第二剂和加强剂BNT162b2疫苗后免疫性血小板减少症复发。

IF 2.4 4区医学

Acta Haematologica Pub Date : 2023-01-01 DOI: 10.1159/000527431

Maja Ratajczak-Enselme, Guy-Clébert Mutoni, Jules Demeules, Laurence Favet, Kuntheavy Ing Lorenzini

引用次数: 2

A Case of Hemophagocytic Lymphohistiocytosis following Second Dose of COVID-19 Vaccination. 第二剂新冠肺炎疫苗接种后发生嗜血球性淋巴组织细胞增多症1例。

IF 2.4 4区医学

Acta Haematologica Pub Date : 2023-01-01 DOI: 10.1159/000526980

Hee Won Park, Gi June Min, Tong Yoon Kim, Seok-Goo Cho

{"title":"A Case of Hemophagocytic Lymphohistiocytosis following Second Dose of COVID-19 Vaccination.","authors":"Hee Won Park, Gi June Min, Tong Yoon Kim, Seok-Goo Cho","doi":"10.1159/000526980","DOIUrl":"https://doi.org/10.1159/000526980","url":null,"abstract":"Hemophagocytic lymphohistiocytosis (HLH) is a rare, severe hyperinflammatory disease characterized by overproduction of cytokines and hemophagocytosis of hematopoietic cells, resulting in multiorgan failure. Prompt treatment initiation is essential for patient survival. The coronavirus disease 2019 (COVID-19) pandemic has led to the rapid development of several vaccines, including BNT162b2 by Pfizer-BioNTech. Few cases of immune-mediated complications of COVID-19 and its vaccines have been reported, characterized by persistent stimulation of the immune system, resembling HLH. We report the case of a 21-year-old man with secondary HLH following a second dose of the BNT162b2 vaccine. The patient did not have primary HLH or other contributors to secondary HLH and met the HLH-2004 diagnostic criteria. He was safely treated with steroid pulse therapy alone, without etoposide, cyclosporin, or immunoglobulins, which are recommended for pediatric patients. Physicians need to be aware of such severe complications following a second dose of the COVID-19 vaccine.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":"146 1","pages":"65-71"},"PeriodicalIF":2.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9747738/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10666225","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: 2

IRF4 Gene Expression on the Trail of Molecular Response: Looking at Chronic Myeloid Leukemia from Another Perspective. IRF4基因在分子反应过程中的表达:从另一个角度看慢性髓系白血病。

IF 2.4 4区医学

Acta Haematologica Pub Date : 2023-01-01 DOI: 10.1159/000527173

Francesco Tarantini, Cosimo Cumbo, Elisa Parciante, Luisa Anelli, Antonella Zagaria, Nicoletta Coccaro, Crescenzio Francesco Minervini, Giuseppina Tota, Immacolata Redavid, Maria Rosa Conserva, Immacolata Attolico, Antonella Russo Rossi, Giorgina Specchia, Pellegrino Musto, Francesco Albano

{"title":"IRF4 Gene Expression on the Trail of Molecular Response: Looking at Chronic Myeloid Leukemia from Another Perspective.","authors":"Francesco Tarantini, Cosimo Cumbo, Elisa Parciante, Luisa Anelli, Antonella Zagaria, Nicoletta Coccaro, Crescenzio Francesco Minervini, Giuseppina Tota, Immacolata Redavid, Maria Rosa Conserva, Immacolata Attolico, Antonella Russo Rossi, Giorgina Specchia, Pellegrino Musto, Francesco Albano","doi":"10.1159/000527173","DOIUrl":"https://doi.org/10.1159/000527173","url":null,"abstract":"Introduction: Interferon regulatory factor 4 (IRF4) is a transcriptional factor with a key role in the modulation of inflammation and immune surveillance. The IRF4 gene is downregulated in Philadelphia-negative myeloproliferative neoplasms, and its expression is associated with prognosis and response to treatment.Methods: We evaluated the IRF4 expression kinetics during tyrosine kinase inhibitor (TKI) treatment in a cohort of 116 chronic myeloid leukemia (CML) patients to elucidate its role in the disease course.Results: A relationship between the IRF4 expression and the disease burden was observed at various disease stages. A correlation analysis between the International Scale (IS) and IRF4 values confirmed this close association. A significant increase is detected after 3 months of TKI treatment. Patients achieving an early molecular response (EMR) had higher IRF4 values at both diagnosis and after 3 months of therapy as compared to those failing the EMR target. Patients achieving treatment-free remission did not show IRF4 fluctuations during monitoring, while a decreased IRF4 expression emerged at the time of molecular relapse.Conclusion: Our data seem to confirm the relevance of IRF4 in the pathogenesis of CML, suggesting a pivotal role at the disease onset and a predictive value during the CML course.","PeriodicalId":6981,"journal":{"name":"Acta Haematologica","volume":"146 1","pages":"37-43"},"PeriodicalIF":2.4,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10666259","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: 1