Hematology, transfusion and cell therapy最新文献

{"title":"Transcriptional regulators of fetal hemoglobin.","authors":"Gabriela Pereira Dos Santos, Larissa Teodoro Rabi, André Alves Bezerra, Marcelo Rodrigues da Cunha, Amilton Iatecola, Victor Augusto Ramos Fernandes","doi":"10.1016/j.htct.2024.06.001","DOIUrl":"https://doi.org/10.1016/j.htct.2024.06.001","url":null,"abstract":"<p><p>Sickle cell anemia is a hereditary disease caused by sickle-shaped red blood cells that can lead to vaso-occlusive crises. Treatment options are currently limited, highlighting the need to develop new clinical approaches. Studies demonstrated that elevated levels of fetal hemoglobin (Hb F) are associated with a reduction of mortality and morbidity in sickle cell anemia patients. In light of this, researchers have been trying to elucidate the transcriptional regulation of Hb F to develop new therapeutic interventions. The present study aimed to present the main transcription factors of Hb F and discuss the clinical feasibility of these molecular targets. Two search strategies were used in the PubMed, SciELO, and LILACS databases between July and August 2023 to conduct this review. Manual searches were also conducted by checking references of potentially eligible studies. Eligibility criteria consisted of clinical trials and cohort studies from the last five years that investigated transcription factors associated with Hb F. The transcription factors investigated in at least four eligible studies were included in this review. As a result, 56 eligible studies provided data on the BCL11A, LRF, NF-Y, GATA1, KLF1, HRI, ATF4, and MYB factors. The studies demonstrated that Hb F is cooperatively regulated by transcription factors with the BCL11A factor appearing to be the most specific target gene for γ-globin induction. Although these data are promising, there are still significant gaps and intervention limitations due to the adverse functions of the target genes. New studies that clarify the aspects and functionalities of Hb F regulators may enable new clinical approaches for sickle cell anemia patients.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142142130","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Chronic lymphocytic leukemia diagnosis: how many more algorithms and scoring systems do we need?","authors":"Daniel Mazza Matos","doi":"10.1016/j.htct.2024.05.007","DOIUrl":"https://doi.org/10.1016/j.htct.2024.05.007","url":null,"abstract":"","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-08-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142094237","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Patient-reported outcomes of treatment and adverse effects following acute lymphoblastic leukemia: a low- and middle-income country cross-sectional study.","authors":"Eduardo Cerello Chapchap, Nina Melo, Denise Martins, Maria Lucia Lee, Nelson Hamerschlak","doi":"10.1016/j.htct.2024.05.006","DOIUrl":"https://doi.org/10.1016/j.htct.2024.05.006","url":null,"abstract":"<p><strong>Introduction: </strong>The scenario of adult patients with acute lymphoblastic leukemia treated in Brazil has not been well described yet.</p><p><strong>Methods: </strong>Four hundred patients diagnosed with acute lymphoblastic leukemia from 1981 to 2019, registered in the Brazilian lymphoma and leukemia association (ABRALE) or their caregivers were interviewed by telephone to evaluate patient-reported perceptions of diagnosis, treatment and adverse effects.</p><p><strong>Results: </strong>Overall, 203 were male with a mean age of 15.7 years and median follow-up of 6.2 years. Main presenting symptoms were fever (39 %), bleeding/ecchymosis (38 %), intense fatigue (30 %), and musculoskeletal pain (28 %). The proportion of patients diagnosed within one week of symptoms onset differed between public (17.9 %) and private healthcare (31.1 %; p-value = 0.019). Additionally, diagnostic difficulties were higher in public care: 35 % versus 22.6 % (p-value = 0.034). Only 36 patients were able to report their treatment protocols; from a list of eight reported protocols, the most common were the Brazilian Childhood Cooperative Group for Treatment of Acute Lymphoblastic Leukemia in Children (GBTLI - 10/27.8 %) and Berlin-Frankfurt-Münster (BFM - 8/22.2 %). Seventy patients (17.5 %) required treatment modification, 37.1 % due to severe adverse effects; 21.7 % received short treatment duration (≤6 months) and 16 % proceeded to allogeneic hematopoietic stem cell transplantation with 17/64 (27 %) reporting difficulties in this step, characterized as >3 months delay. Indication for transplantation was related to minimal residual disease and cranial radiotherapy; 41.7 % reported treatment-related adverse effects (range: 1-6), in particular: mood disorders (26.3 %), neurologic deficit (13.8 %), cognitive/memory impairment (12 %), and lung disease (15 %). Risk factors for adverse effects were age, indication of transplantation and living in a large city. Treatment disparities such as diagnostic and transplantation delays remain challenges in these patients.</p><p><strong>Conclusions: </strong>Urgent interventions are needed to optimize healthcare and reduce adverse effects, especially in adolescent and young adult patients.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141918359","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cytokine release syndrome after chimeric antigen receptor T cell therapy in patients with diffuse large B-cell lymphoma: a systematic review","authors":"Andressa Rodrigues dos Santos, Daniela Zanini, Daniel Andolfatto","doi":"10.1016/j.htct.2024.05.005","DOIUrl":"https://doi.org/10.1016/j.htct.2024.05.005","url":null,"abstract":"","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141849108","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Treatment with low-dose tyrosine kinase inhibitors due to significant haematologic toxicity in patients with CML with prolonged treatment failure prevents haematologic progression","authors":"Lucia Vráblová, Hana Klamova, I. Skoumalová, Jana Navrátilová, R. Janská, Janine Grohmann, Milena Holzerová, Edgar Faber","doi":"10.1016/j.htct.2024.03.010","DOIUrl":"https://doi.org/10.1016/j.htct.2024.03.010","url":null,"abstract":"","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141851439","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diffuse mucocutaneous hyperpigmentation related to hydroxyurea","authors":"Vishnu Sharma, Sidharth Mahajan, Vansh Bagrodia","doi":"10.1016/j.htct.2024.03.009","DOIUrl":"https://doi.org/10.1016/j.htct.2024.03.009","url":null,"abstract":"","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141840759","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IKZF1 and BTG1 silencing reduces glucocorticoid response in B-cell precursor acute leukemia cell line","authors":"Amanda de Albuquerque, Bruno A. Lopes, Renan Amphilophilo Fernandes, E. Gimba, M. Emerenciano","doi":"10.1016/j.htct.2024.05.004","DOIUrl":"https://doi.org/10.1016/j.htct.2024.05.004","url":null,"abstract":"","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141850740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of autologous bone marrow transplantation in primary effusion lymphoma: a case report and literature review","authors":"Vitor Abreu de Goes, A. Cortez, Diogo Lago Morbeck, Felipe D’Almeida Costa, Talita Máira Bueno da Silveira","doi":"10.1016/j.htct.2024.04.119","DOIUrl":"https://doi.org/10.1016/j.htct.2024.04.119","url":null,"abstract":"","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141842553","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Relationship between response to first-line steroid treatment in adult immune thrombocytopenic purpura and the course of the disease","authors":"Esra Seçkin","doi":"10.1016/j.htct.2024.03.008","DOIUrl":"https://doi.org/10.1016/j.htct.2024.03.008","url":null,"abstract":"","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141841699","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pregnant women's knowledge and clinical management of hemolytic disease of the fetus and newborn in Pará, Brazil.","authors":"Jhemily de Nazaré Gonçalves E Silva, Andrya Maia de Souza, Fabiane Monteiro do Rosario, Laine Celestino Pinto","doi":"10.1016/j.htct.2024.03.007","DOIUrl":"https://doi.org/10.1016/j.htct.2024.03.007","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the knowledge of pregnant women and the clinical management of hemolytic disease of the fetus and newborn, as well as to describe the gestational profile, risk factors and socio-epidemiological profile of pregnant women treated at two municipal health units in Belém (Pará, Brazil).</p><p><strong>Methods: </strong>This was a cross-sectional analytical study, which consisted in the application of questionnaires to pregnant women who underwent prenatal care at the municipal health units.</p><p><strong>Results: </strong>A total of 104 pregnant women were evaluated; most were aged between 24 and 29 years old, had high school degrees (38 %), family incomes between 1 and 2 minimum wages (45 %) and blood type O+ (43 %). Regarding the gestational profile, the participants were predominantly in the third trimester of pregnancy (49 %), started prenatal care in the first gestational trimester (81 %) and were primiparous (61 %). Failures in the management of prenatal care were observed, especially with regard to access to information about the disease, since most pregnant women did not receive information about blood incompatibility during prenatal care. This led to limited knowledge about the pathology of the disease evidenced by the fact that most of the correct answers were between Questions 0-4, which were significantly associated with the women's education and income.</p><p><strong>Conclusions: </strong>Although hemolytic disease of the fetus and newborn is serious, the pregnant women in this study demonstrated little knowledge about the disease and had inadequate care by health professionals, reinforcing the importance of improving care for women's health and prenatal care.</p>","PeriodicalId":94026,"journal":{"name":"Hematology, transfusion and cell therapy","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-05-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141263578","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}