Revista Brasileira de Hematologia e Hemoterapia最新文献

{"title":"Identification of the MYST3-CREBBP fusion gene in infants with acute myeloid leukemia and hemophagocytosis","authors":"Francianne Gomes Andrade ,&nbsp;Elda Pereira Noronha ,&nbsp;Rosania Maria Baseggio ,&nbsp;Teresa Cristina Cardoso Fonseca ,&nbsp;Bruno Marcelo Rocha Freire ,&nbsp;Isis M. Quezado Magalhaes ,&nbsp;Ilana R. Zalcberg ,&nbsp;Maria S. Pombo-de-Oliveira","doi":"10.1016/j.bjhh.2016.06.005","DOIUrl":"10.1016/j.bjhh.2016.06.005","url":null,"abstract":"<div><h3>Background</h3><p>Acute myeloid leukemia presenting the <em>MYST3-CREBBP</em> fusion gene is a rare subgroup associated with hemophagocytosis in early infancy and monocytic differentiation. The aim of this study was to define the relevant molecular cytogenetic characteristics of a unique series of early infancy acute myeloid leukemia cases (≤24<!--> <!-->months old), based on the presence of hemophagocytosis by blast cells at diagnosis.</p></div><div><h3>Methods</h3><p>A series of 266 infant cases of acute myeloid leukemia was the reference cohort for the present analysis. Acute myeloid leukemia cases with hemophagocytosis by blast cells were reviewed to investigate the presence of the <em>MYST3-CREBBP</em> fusion gene by fluorescence <em>in situ</em> hybridization (FISH) and reverse transcription polymerase chain reaction.</p></div><div><h3>Results</h3><p>Eleven cases with hemophagocytosis were identified with hemophagocytic lymphohistiocytosis being ruled out. Six cases were classified as myelomonocytic leukemia, three as AML-M7 and two as AML-M2. In five cases, the presence of the <em>MYST3-CREBBP</em> fusion gene identified by molecular cytogenetics was confirmed by fluorescence <em>in situ</em> hybridization. All patients received treatment according to the Berlin–Frankfürt–Münster acute myeloid leukemia protocols and only one out of the five patients with the <em>MYST3-CREBBP</em> fusion gene is still alive.</p></div><div><h3>Conclusions</h3><p>Our findings demonstrate that the presence of hemophagocytosis in acute myeloid leukemia was not exclusively associated to the <em>MYST3-CREBBP</em> fusion gene. Improvements in molecular cytogenetics may help to elucidate more complex chromosomal rearrangements in infants with acute myeloid leukemia and hemophagocytosis.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 291-297"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.06.005","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54246004","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":"Guidelines on Beta-thalassemia major – regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira – 2016","authors":"Dante Langhi Jr ,&nbsp;Eugênia Maria Amorim Ubiali ,&nbsp;José Francisco Comenalli Marques Jr ,&nbsp;Mônica de Almeida Verissimo ,&nbsp;Sandra Regina Loggetto ,&nbsp;Antonio Silvinato ,&nbsp;Wanderley Marques Bernardo","doi":"10.1016/j.bjhh.2016.09.003","DOIUrl":"10.1016/j.bjhh.2016.09.003","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 341-345"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.09.003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54246316","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":"Hematopoietic stem cell mobilization for autologous transplantation in multiple myeloma patients previously exposed to cyclophosphamide, thalidomide, and dexamethasone: is granulocyte-colony stimulating factor alone enough?","authors":"Afonso Celso Vigorito","doi":"10.1016/j.bjhh.2016.07.006","DOIUrl":"10.1016/j.bjhh.2016.07.006","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 281-282"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.07.006","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54245746","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":"Outcomes of allogeneic hematopoietic stem cell transplantation for lymphomas: a single-institution experience","authors":"Mira Romany Massoud ,&nbsp;Paolo Fabrizio Caimi ,&nbsp;Nicole Ferrari ,&nbsp;Pingfu Fu ,&nbsp;Richard Creger ,&nbsp;Robert Fox ,&nbsp;Joanne Carlson-Barko ,&nbsp;Merle Kolk ,&nbsp;Lauren Brister ,&nbsp;Brenda Wimpfheimer Cooper ,&nbsp;Stanton Gerson ,&nbsp;Hillard Michael Lazarus ,&nbsp;Marcos de Lima ,&nbsp;Basem Magdy William","doi":"10.1016/j.bjhh.2016.07.003","DOIUrl":"10.1016/j.bjhh.2016.07.003","url":null,"abstract":"<div><h3>Introduction</h3><p>Allogeneic hematopoietic stem cell transplantation offers the opportunity for extended survival in patients with Hodgkin's and non-Hodgkin lymphomas who relapsed after, or were deemed ineligible for, autologous transplantation. This study reports the cumulative experience of a single center over the past 14 years aiming to define the impact of patient, disease, and transplant-related characteristics on outcomes.</p></div><div><h3>Methods</h3><p>All patients with histologically confirmed diagnosis of Hodgkin's or non-Hodgkin lymphomas who received allogeneic transplantation from 2000 to 2014 were retrospectively studied.</p></div><div><h3>Results</h3><p>Forty-one patients were reviewed: 10 (24%) had Hodgkin's and 31 (76%) had non-Hodgkin lymphomas. The median age was 50 years and 23 (56%) were male. The majority of patients (68%) had had a prior autologous transplantation. At the time of allogeneic transplantation, 18 (43%) patients were in complete and seven (17%) were in partial remission. Most (95%) patients received reduced-intensity conditioning, 49% received matched sibling donor grafts, 24% matched-unrelated donor grafts, and 27% received double umbilical cord blood grafts. The 100-day treatment-related mortality rate was 12%. After a median duration of follow up of 17.1 months, the median progression-free and overall survival was 40.5 and 95.8 months, respectively. On multivariate analysis, patients who had active disease at the time of transplant had inferior survival.</p></div><div><h3>Conclusions</h3><p>Allogeneic transplantation results extend survival in selected patients with relapsed/refractory Hodgkin's and non-Hodgkin lymphomas with low treatment-related mortality. Patients who have active disease at the time of allogeneic transplantation have poor outcomes.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 314-319"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.07.003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54246122","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":"Survival and treatment response in adults with acute promyelocytic leukemia treated with a modified International Consortium on Acute Promyelocytic Leukemia protocol","authors":"Erick Crespo-Solis,&nbsp;Jorge Contreras-Cisneros,&nbsp;Roberta Demichelis-Gómez,&nbsp;Adriana Rosas-López,&nbsp;Juan Mauricio Vera-Zertuche,&nbsp;Alvaro Aguayo,&nbsp;Xavier López-Karpovitch","doi":"10.1016/j.bjhh.2016.08.002","DOIUrl":"10.1016/j.bjhh.2016.08.002","url":null,"abstract":"<div><p>Acute promyelocytic leukemia has good prognosis in view of the high complete remission and survival rates achieved with therapies containing all-trans retinoic acid or arsenic trioxide. However, there is a significant risk of death during induction due to hemorrhage secondary to disseminated intravascular coagulation. This has contributed to a gap in the prognosis of patients between developed and developing countries. The International Consortium on Acute Promyelocytic Leukemia was created in 2005 and proposed a treatment protocol based on daunorubicin and all-trans retinoic acid stratified by risk geared toward developing countries. Herein are presented the results from the first patient cohort treated in a single developing country hospital employing a slightly modified version of the International Consortium protocol in a real life setting. Twenty patients with acute promyelocytic leukemia were enrolled: 27.8% had low-risk, 55.6% intermediate risk and 16.7% high-risk. The complete remission rate was 94.4% after a median of 42 days. Both relapse rates and death rates were one patient (5.5%) each. No deaths were observed during consolidation. After a median follow-up of 29 months, the overall survival rate was 89.1%. Efficacy and safety of the International Consortium on Acute Promyelocytic Leukemia protocol has been reproduced in acute promyelocytic leukemia patients from a developing country.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 285-290"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.08.002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54245825","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":"Is it feasible to use granulocyte-colony stimulating factor alone to mobilize progenitor cells in multiple myeloma patients induced with a cyclophosphamide, thalidomide and dexamethasone regimen?","authors":"Edvan de Queiroz Crusoe ,&nbsp;Fabiana Higashi ,&nbsp;Gracia Aparecida Martinez ,&nbsp;José Carlos Barros ,&nbsp;Marcelo Bellesso ,&nbsp;Marina Rossato ,&nbsp;Ana Cinira F. Marret ,&nbsp;Carlos Sérgio Chiattone ,&nbsp;Vania Tietsch de Moraes Hungria","doi":"10.1016/j.bjhh.2016.06.004","DOIUrl":"10.1016/j.bjhh.2016.06.004","url":null,"abstract":"<div><h3>Background</h3><p>Cyclophosphamide plus thalidomide as induction for multiple myeloma patients eligible for autologous stem cell transplantation may be a limiting factor for cell mobilization. The minimum acceptable mobilized peripheral blood stem cell count to prevent deleterious effects during transplantation is 2.0<!--> <!-->×<!--> <!-->10⁶ CD34⁺ cells/kg. Combining other treatments to granulocyte-colony stimulating factor, such as cyclophosphamide, could overcome the mobilization limitation. The objective of this study was to assess the number of CD34⁺ cells mobilized using granulocyte-colony stimulating factor with and without cyclophosphamide after induction with cyclophosphamide, thalidomide and dexamethasone.</p></div><div><h3>Methods</h3><p>A retrospective study was performed of a cohort of multiple myeloma patients submitted to autologous stem cell transplantations at two Brazilian centers between May 2009 and July 2013. The oral cyclophosphamide and thalidomide induction doses used were 1500<!--> <!-->mg/month and 100–200<!--> <!-->mg/day, respectively. Mobilization doses were 10–15<!--> <!-->mcg/kg granulocyte-colony stimulating factor with 2–4<!--> <!-->g/m² cyclophosphamide, or 15–20<!--> <!-->mcg/kg granulocyte-colony stimulating factor alone for 5 days. Collection of &gt;2.0<!--> <!-->×<!--> <!-->10⁶ CD34⁺ cells/kg was considered sufficient.</p></div><div><h3>Results</h3><p>Eighty-eight patients were analyzed; only 18 received cyclophosphamide. The median age was 58 years old (range: 51–62) for the granulocyte-colony stimulating factor group and 56.5 years old (range: 54–60) for granulocyte-colony stimulating factor plus cyclophosphamide group. Fifty-two patients were male. Eighty cases (90.9%) were Durie-Salmon Staging System III-A/B and 38 (44.7%) and 20 cases (23.5%) were International Staging System 2 and 3, respectively. The group that received cyclophosphamide collected a higher median number of progenitor cells [3.8 (range: 3.1–4.4) vs. 3.2 (range: 2.3–3.8)] (<em>p</em>-value<!--> <!-->=<!--> <!-->0.008). No correlation was observed between better responses or number of induction cycles and the number of cells collected.</p></div><div><h3>Conclusion</h3><p>The number of cells mobilized with granulocyte-colony stimulating factor plus cyclophosphamide was higher. However, in both groups, the median number of CD34⁺ cells was sufficient to perform a single autologous stem cell transplantation; no deleterious effects were reported during harvesting.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 302-309"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.06.004","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54245969","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 closer look into blood group discrepancy arising due to an underlying malignancy","authors":"Rajeswari Subramaniyan ,&nbsp;Balan Louis Gaspar","doi":"10.1016/j.bjhh.2016.04.007","DOIUrl":"10.1016/j.bjhh.2016.04.007","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 361-363"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.04.007","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54245393","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 clinical challenge: Treatment of acute myeloid leukemia in a Jehovah's Witness","authors":"Daniela Cárdenas-Araujo,&nbsp;Elías Eugenio González-López,&nbsp;Xitlaly Judith González-Leal,&nbsp;José Carlos Jaime-Pérez,&nbsp;David Gómez-Almaguer","doi":"10.1016/j.bjhh.2016.05.003","DOIUrl":"10.1016/j.bjhh.2016.05.003","url":null,"abstract":"","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 358-360"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.05.003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54245407","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":"Herpes zoster after autologous hematopoietic stem cell transplantation","authors":"Kelli Borges dos Santos,&nbsp;Rafaela Souto e Souza,&nbsp;Angelo Atalla,&nbsp;Abrahão Elias Hallack-Neto","doi":"10.1016/j.bjhh.2016.05.015","DOIUrl":"10.1016/j.bjhh.2016.05.015","url":null,"abstract":"<div><h3>Background</h3><p>The autologous hematopoietic stem cell transplantation procedure involves immunosuppression of the patient. Thus, the patient has an elevated risk for several diseases, such as infections with the varicella-zoster virus. Prevention protocols have been proposed based on the use of acyclovir from the first day of conditioning, and maintaining this drug for 30–100 days after the procedure or for as much as one year. The objective of this work was to evaluate the incidence of herpes zoster after autologous transplantations related to the early suspension of acyclovir.</p></div><div><h3>Methods</h3><p>A retrospective study was carried out based on the collection of data from 231 medical records of transplant patients in the Bone Marrow Transplant Unit of the teaching hospital of the Universidade Federal de Juiz de Fora in the period between 2004 and 2014.</p></div><div><h3>Results</h3><p>Fourteen (6.1%) patients had herpes zoster in the post-transplant period on average within six months of the procedure. Patients with multiple myeloma (64.3%) were the most affected. There was a statistically significant difference in the age of the patients, with older individuals having a greater chance of developing the infection (<em>p</em>-value<!--> <!-->=<!--> <!-->0.002). There were no significant differences for the other variables analyzed.</p></div><div><h3>Conclusion</h3><p>The early suspension of acyclovir can be safe in patients who receive autologous hematopoietic stem cell transplants. However some groups may benefit from extended prophylaxis with acyclovir, particularly older patients and patients with multiple myeloma.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 298-301"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.05.015","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54245421","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":"Structural diversity and biological importance of ABO, H, Lewis and secretor histo-blood group carbohydrates","authors":"Luiz Carlos de Mattos","doi":"10.1016/j.bjhh.2016.07.005","DOIUrl":"10.1016/j.bjhh.2016.07.005","url":null,"abstract":"<div><p>ABO, H, secretor and Lewis histo-blood system genes control the expression of part of the carbohydrate repertoire present in areas of the body occupied by microorganisms. These carbohydrates, besides having great structural diversity, act as potential receptors for pathogenic and non-pathogenic microorganisms influencing susceptibility and resistance to infection and illness. Despite the knowledge of some structural variability of these carbohydrate antigens and their polymorphic levels of expression in tissue and exocrine secretions, little is known about their biological importance and potential applications in medicine. This review highlights the structural diversity, the biological importance and potential applications of ABO, H, Lewis and secretor histo-blood carbohydrates.</p></div>","PeriodicalId":21233,"journal":{"name":"Revista Brasileira de Hematologia e Hemoterapia","volume":"38 4","pages":"Pages 331-340"},"PeriodicalIF":0.0,"publicationDate":"2016-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.bjhh.2016.07.005","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54245695","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}