Revista Brasileira de Hematologia e Hemoterapia最新文献

{"title":"Detection of Human Adenovirus (species-C, -D and -F) in an allogeneic stem cell transplantation recipient: a case report","authors":"Hugo César Pereira Santos , Francielly Pinheiro da Silva Borges , Adriano de Moraes Arantes , Menira Souza","doi":"10.1016/j.bjhh.2016.09.006","DOIUrl":"10.1016/j.bjhh.2016.09.006","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 60-62"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.09.006","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A combination of the -α3.7 and --MEDII alleles causing hemoglobin H disease in a Brazilian patient","authors":"Roberta Dorta Ferreira, Natália de Oliveira Mota, Elza Myiuki Kimura, Gisele Audrei Pedroso, Maria de Fatima Sonati","doi":"10.1016/j.bjhh.2016.12.001","DOIUrl":"10.1016/j.bjhh.2016.12.001","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 80-83"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.12.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791703","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Phase Ib open label, randomized, safety study of SANGUINATE™ in patients with sickle cell anemia","authors":"Hemant Misra ,&nbsp;James Bainbridge ,&nbsp;John Berryman ,&nbsp;Abraham Abuchowski ,&nbsp;Kenneth Mauricio Galvez ,&nbsp;Luis Fernando Uribe ,&nbsp;Angel Luis Hernandez ,&nbsp;Nestor Rodolfo Sosa","doi":"10.1016/j.bjhh.2016.08.004","DOIUrl":"10.1016/j.bjhh.2016.08.004","url":null,"abstract":"<div><h3>Background</h3><p>Treatment of sickle cell anemia is a challenging task and despite the well understood genetic and biochemical pathway of sickle hemoglobin, current therapy continues to be limited to the symptomatic treatment of pain, supplemental oxygen, antibiotics, red blood cell transfusions and hydroxyurea. SANGUINATE is a carbon monoxide releasing molecule and oxygen transfer agent under clinical development for the treatment of sickle cell anemia and comorbidities.</p></div><div><h3>Methods</h3><p>An open-label randomized Phase Ib study was performed in adult sickle cell anemia patients. Two dose levels of SANGUINATE were compared to hydroxyurea in 24 homozygotes for Hb SS. Twelve subjects received either a low dose (160<!--> <!-->mg/kg) of SANGUINATE or 15<!--> <!-->mg/kg hydroxyurea. Another 12 subjects received either a high dose (320<!--> <!-->mg/kg) of SANGUINATE or 15<!--> <!-->mg/kg hydroxyurea. The primary endpoint was the safety of SANGUINATE <em>versus</em> hydroxyurea in sickle cell anemia patients. Secondary endpoints included determination of the plasma pharmacokinetics and assessment of hematologic measurements.</p></div><div><h3>Results</h3><p>Musculoskeletal related adverse events were the most common. Transient troponin I levels increased in three patients, one of whom had an increase in tricuspid regurgitant velocity; however, no clinical signs were noted. Following an assessment of vital signs, tricuspid regurgitant velocity, electrocardiogram, serum biochemistry, hematology, urinalysis, and analysis of reported adverse events, SANGUINATE was found to be safe in stable sickle cell anemia patients.</p></div><div><h3>Conclusions</h3><p>The clinical trial met its primary objective of demonstrating an acceptable safety profile for SANGUINATE in patients with sickle cell anemia. This trial established the safety of SANGUINATE at both dose levels and permitted its advance to Phase II trials.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 20-27"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.08.004","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791797","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comprehensive neuropsychological evaluation of children and adolescents with sickle cell anemia: a hospital-based sample","authors":"Samantha Nunes,&nbsp;Nayara Argollo,&nbsp;Marivania Mota,&nbsp;Camilo Vieira,&nbsp;Eduardo Pondé de Sena","doi":"10.1016/j.bjhh.2016.09.004","DOIUrl":"10.1016/j.bjhh.2016.09.004","url":null,"abstract":"<div><h3>Background</h3><p>Individuals with sickle cell anemia may suffer symptomatic or silent cerebral infarcts leading to neurocognitive complications. This study investigated the cognitive and intellectual performance of children and adolescents with sickle cell anemia.</p></div><div><h3>Methods</h3><p>The socioeconomic status, clinical aspects and behavioral profile of 15 young individuals with sickle cell anemia were evaluated. The Wechsler Intelligence Scale for Children, the Developmental Neuropsychological Assessment Test, and the Child Behavior Checklist were applied.</p></div><div><h3>Results</h3><p>Participants with a history of stroke had lower intelligence quotient (IQ) scores. Alterations were found in attention and executive functioning, language, verbal and visual memory, visuospatial processing and sensorimotor skills. These alterations were found both in the children and adolescents who had had a cerebral infarction and in those who apparently had not. In the majority of cases, there were learning difficulties, a history of repeating school years and a need for specialist educational support. The most common additional diagnoses in accordance with the Diagnostic and Statistical Manual of Mental Disorders IV were depressive disorder, anxiety disorder and somatic disorder, as well as conditions associated with physical and psychosocial repercussions of sickle cell anemia.</p></div><div><h3>Conclusion</h3><p>As sickle cell anemia is considered a progressive cerebral vasculopathy, it is a potential risk factor for neurocognitive and psychosocial development. Therefore, periodic neuropsychological and behavioral evaluations of children and adolescents with sickle cell anemia may represent a useful measure to reduce long-term biopsychosocial repercussions.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 32-39"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.09.004","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791799","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical and laboratory profile of patients with sickle cell anemia","authors":"Phelipe Gabriel dos Santos Sant’Ana ,&nbsp;Ariane Moreira Araujo ,&nbsp;Cynthia Teixeira Pimenta ,&nbsp;Mário Lúcio Pacheco Ker Bezerra ,&nbsp;Sílvio Pereira Borges Junior ,&nbsp;Viviana Martins Neto ,&nbsp;Janaina Sousa Dias ,&nbsp;Aline de Freitas Lopes ,&nbsp;Danyelle Romana Alves Rios ,&nbsp;Melina de Barros Pinheiro","doi":"10.1016/j.bjhh.2016.09.007","DOIUrl":"10.1016/j.bjhh.2016.09.007","url":null,"abstract":"<div><h3>Objective</h3><p>This study aimed to describe and analyze clinical and laboratory characteristics of patients with sickle cell anemia treated at the Hemominas Foundation, in Divinópolis, Brazil. Furthermore, this study aimed to compare the clinical and laboratory outcomes of the group of patients treated with hydroxyurea with those patients that were not treated with hydroxyurea.</p></div><div><h3>Methods</h3><p>Clinical and laboratorial data were obtained by analyzing medical records of patients with sickle cell anemia.</p></div><div><h3>Results</h3><p>Data from the medical records of 50 patients were analyzed. Most of the patients were female (56%), aged between 20 and 29 years old. Infections, transfusions, cholecystectomy, splenectomy and systemic arterial hypertension were the most common clinical adverse events of the patients. The most frequent cause of hospitalization was painful crisis. The majority of patients had reduced values of hemoglobin and hematocrit (8.55<!--> <!-->±<!--> <!-->1.33<!--> <!-->g/dL and 25.7<!--> <!-->±<!--> <!-->4.4%, respectively) and increased fetal hemoglobin levels (12<!--> <!-->±<!--> <!-->7%). None of the clinical variables was statistically significant on comparing the two groups of patients. Among hematological variables only hemoglobin and hematocrit levels were statistically different between patients treated with hydroxyurea and untreated patients (<em>p</em>-value<!--> <!-->=<!--> <!-->0.005 and <em>p</em>-value<!--> <!-->=<!--> <!-->0.001, respectively).</p></div><div><h3>Conclusion</h3><p>Sickle cell anemia requires treatment and follow-up by a multiprofessional team. A current therapeutic option is hydroxyurea. This drug reduces complications and improves laboratorial parameters of patients. In this study, the use of the drug increased the hemoglobin and hematocrit levels of patients.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 40-45"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.09.007","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791802","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Erratum to “Diagnosis and treatment of chronic lymphocytic leukemia: Recommendations from the Brazilian Group of Chronic Lymphocytic Leukemia” [Rev Bras Hematol Hemoter. 2016;38(4):346–357]","authors":"Celso Arrais Rodrigues ,&nbsp;Matheus Vescovi Gonçalves ,&nbsp;Maura Rosane Valério Ikoma ,&nbsp;Irene Lorand-Metze ,&nbsp;André Domingues Pereira ,&nbsp;Danielle Leão Cordeiro de Farias ,&nbsp;Maria de Lourdes Lopes Ferrari Chauffaille ,&nbsp;Rony Schaffel ,&nbsp;Eduardo Flávio Oliveira Ribeiro ,&nbsp;Talita Silveira da Rocha ,&nbsp;Valeria Buccheri ,&nbsp;Yuri Vasconcelos ,&nbsp;Vera Lúcia de Piratininga Figueiredo ,&nbsp;Carlos Sérgio Chiattone ,&nbsp;Mihoko Yamamoto ,&nbsp;on behalf of the Brazilian Group of Chronic Lymphocytic Leukemia","doi":"10.1016/j.bjhh.2017.01.002","DOIUrl":"10.1016/j.bjhh.2017.01.002","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 93-94"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2017.01.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791709","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mantle cell lymphoma presenting as multiple lymphomatous polyposis of the gastrointestinal tract","authors":"Cláudio Martins,&nbsp;Cristina Teixeira,&nbsp;Élia Gamito,&nbsp;Ana Paula Oliveira","doi":"10.1016/j.bjhh.2016.11.005","DOIUrl":"10.1016/j.bjhh.2016.11.005","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 73-76"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.11.005","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791702","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary breast lymphoma: a case report","authors":"María Mar Herráez-Albendea,&nbsp;María Castillo Jarilla-Fernández,&nbsp;Francisco Javier Jiménez-Burgos","doi":"10.1016/j.bjhh.2016.09.011","DOIUrl":"10.1016/j.bjhh.2016.09.011","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 89-90"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.09.011","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791705","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mortality by sickle cell disease in Brazil","authors":"Giovanna Abadia Oliveira Arduini ,&nbsp;Letícia Pinto Rodrigues ,&nbsp;Alessandra Bernadete Trovó de Marqui","doi":"10.1016/j.bjhh.2016.09.008","DOIUrl":"10.1016/j.bjhh.2016.09.008","url":null,"abstract":"<div><p>This work aimed to characterize mortality by sickle cell disease in Brazil. The MEDLINE electronic database was searched using the terms ‘mortality’ and ‘sickle cell disease’ and ‘Brazil’ for articles published in the last five years aiming to provide a current analysis of the subject in question. Eight studies on mortality by sickle cell disease were carried out in the Brazilian states of Maranhão, Bahia, Minas Gerais, Rio de Janeiro and Mato Grosso do Sul. The majority of the deaths occurred in patients with sickle cell anemia, which is the most common genotype and causes the most severe clinical manifestation of the disease. In summary, there are few published studies on mortality related to sickle cell disease in Brazil, and most are from the state of Minas Gerais. This study emphasizes the importance of developing more studies on sickle cell disease mortality, so that it may be possible to profile gene carriers and give health professionals more data to strategize the delivery of more effective assistance to these individuals. Despite the early diagnosis of sickle cell disease by the Neonatal Screening Program and the use of preventive and therapeutic measures (penicillin, immunization and hydroxyurea), mortality by sickle cell disease on the world stage is still significant.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"39 1","pages":"Pages 52-56"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.09.008","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34791695","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Splicing factor SF3B1 mutations and ring sideroblasts in myelodysplastic syndromes: a Brazilian cohort screening study","authors":"Flávia Sacilotto Donaires ,&nbsp;Felipe Martelli ,&nbsp;Raquel de Melo Alves-Paiva ,&nbsp;Silvia Maria Meira Magalhães ,&nbsp;Ronald Feitosa Pinheiro ,&nbsp;Rodrigo Tocantins Calado","doi":"10.1016/j.bjhh.2016.06.002","DOIUrl":"10.1016/j.bjhh.2016.06.002","url":null,"abstract":"<div><h3>Background</h3><p>Myelodysplastic syndromes (MDS) comprise a group of malignant clonal hematologic disorders characterized by ineffective hematopoiesis and propensity for progression to acute myeloid leukemia. Acquired mutations in the gene encoding RNA splicing factor 3B subunit 1 (<em>SF3B1</em>) are highly associated with the MDS subtypes presenting ring sideroblasts, and represent a specific nosological entity. The effects of these mutations on clinical outcomes are diverse and contrasting.</p></div><div><h3>Methods</h3><p>A cohort of 91 Brazilian MDS patients, including patients with ring sideroblasts in the bone marrow, were screened for mutations in the <em>SF3B1</em> hotspots (exons 12–15) by direct Sanger sequencing.</p></div><div><h3>Results</h3><p><em>SF3B1</em> heterozygous mutations were identified in six patients (7%), all of them with ring sideroblasts, thus confirming the association between <em>SF3B1</em> mutations and myelodysplastic syndrome subtypes bearing this morphologic feature (frequency of 6/13, <em>p</em>-value<!--> <!-->&lt;<!--> <!-->0.0001).</p></div><div><h3>Conclusion</h3><p>This is the first screening of <em>SF3B1</em> mutations in a cohort of Brazilian myelodysplastic syndrome patients. Our findings confirm that mutations in this splicing gene correlate with bone marrow ringed sideroblasts.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 320-324"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.06.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54245950","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}