Scielo RSS <![CDATA[Revista Brasileira de Hematologia e Hemoterapia]]> http://www.scielo.br/rss.php?pid=1516-848420160003&lang=en vol. 38 num. 3 lang. en <![CDATA[SciELO Logo]]> http://www.scielo.br/img/en/fbpelogp.gif http://www.scielo.br <![CDATA[Reactive oxygen species overload promotes apoptosis in JAK2V617F-positive cell lines]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300179&lng=en&nrm=iso&tlng=en <![CDATA[Blood group polymorphisms in Brazil]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300182&lng=en&nrm=iso&tlng=en <![CDATA[Bioengineering coagulation factors for improved hemophilia treatments : Comment on: the mutation F309S increases FVIII secretion in human cell line]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300184&lng=en&nrm=iso&tlng=en <![CDATA[Diffuse large B cell gastric lymphoma a rare disease: the effort to obtain scientific data in a multicenter, multinational retrospective trial]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300186&lng=en&nrm=iso&tlng=en <![CDATA[Compound heterozygosity for hemoglobin S and D: what do we need to know?]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300188&lng=en&nrm=iso&tlng=en <![CDATA[Comments on: "Clinical, hematological and genetic data of a cohort of children with hemoglobin SD"]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300190&lng=en&nrm=iso&tlng=en <![CDATA[Dangerous universal donors: the reality of the Hemocentro in Belo Horizonte, Minas Gerais]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300193&lng=en&nrm=iso&tlng=en ABSTRACT BACKGROUND: The term dangerous universal blood donor refers to potential agglutination of the erythrocytes of non-O recipients due to plasma of an O blood group donor, which contains high titers of anti-A and/or anti-B hemagglutinins. Thus, prior titration of anti-A and anti-B hemagglutinins is recommended to prevent transfusion reactions. OBJECTIVE: The aim of this study was to estimate the frequency of dangerous universal donors in the blood bank of Belo Horizonte (Fundação Central de Imuno-Hematologia - Fundação Hemominas - Minas Gerais) by determining the titers of anti-A and anti-B hemagglutinins in O blood group donors. METHOD: A total of 400 O blood group donors were randomly selected, from March 2014 to January 2015. The titers of anti-A and anti-B hemagglutinins (IgM and IgG classes) were obtained using the tube titration technique. Dangerous donors were those whose titers of anti-A or anti-B IgM were ≥128 and/or the titers of anti-A or anti-B IgG were ≥256. Donors were characterized according to gender, age and ethnicity. The hemagglutinins were characterized by specificity (anti-A and anti-B) and antibody class (IgG and IgM). RESULTS: Almost one-third (30.5%) of the O blood group donors were universal dangerous. The frequency among women was higher than that of men (p-value = 0.019; odds ratio: 1.66; 95% confidence interval: 1.08-2.56) and among young donors (18-29 years old) it was higher than for donors between 49 and 59 years old (p-value = 0.015; odds ratio: 3.05; 95% confidence interval: 1.22-7.69). There was no significant association between dangerous universal donors and ethnicity, agglutinin specificity or antibody class. CONCLUSION: Especially platelet concentrates obtained by apheresis (that contain a substantial volume of plasma), coming from dangerous universal donors should be transfused in isogroup recipients whenever possible in order to prevent the occurrence of transfusion reactions. <![CDATA[Frequencies of polymorphisms of the Rh, Kell, Kidd, Duffy and Diego systems of Santa Catarina, Southern Brazil]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300199&lng=en&nrm=iso&tlng=en ABSTRACT BACKGROUND: Red blood cell genes are highly polymorphic with the distribution of alleles varying between different populations and ethnic groups. The objective of this study was to investigate gene polymorphisms of blood groups in the state of Santa Catarina, Southern Brazil. METHODS: Three hundred and seventy-three unrelated blood donors and 31 transfusion-dependent patients were evaluated to investigate polymorphisms of the Rh, Kell, Duffy, Kidd, and Diego blood group systems in a population from the state of Santa Catarina. The subjects, from seven regions that comprise the blood-banking network of the state, were assessed between August 2011 and March 2014. The genotypes of the Rh, Kell, Duffy, Kidd, and Diego systems were determined using the restriction fragment length polymorphism-polymerase chain reaction and allele-specific polymerase chain reaction techniques. RESULTS: The genotype frequencies in this study were significantly different when populations from different regions of Santa Catarina were compared. Furthermore, there were also significant differences in the genetic frequencies compared to other Brazilian states. The genotype frequencies of the Kell and Kidd blood groups are similar to European populations from Naples, Italy and Zurich, Switzerland. CONCLUSION: This article reports for the first time the frequency of polymorphisms of blood group systems in blood donors from Santa Catarina, Southern Brazil. <![CDATA[Distribution of serological screening markers at a large hematology and hemotherapy center in Minas Gerais, Southeastern Brazil]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300206&lng=en&nrm=iso&tlng=en ABSTRACT OBJECTIVE: To assess the distribution of serological markers in blood donors at the blood banks of the Fundação Centro de Hematologia e Hemoterapia de Minas Gerais (Hemominas), Brazil, between January 2006 and December 2012. METHODS: This is a descriptive, retrospective study on blood donors screened using serological tests for markers of transmitted diseases at the state blood-banking network. RESULTS: Approximately 78.9% of the donors were considered eligible for the study after clinical screening. Repeat donors represented 68.2% of the total sample, with males being predominant as blood donors (66.8%). Total serological ineligibility was 3.05%, with total anti-HBc being the most common marker (1.26%), followed by syphilis (0.88%) and human immunodeficiency virus (0.36%). The prevalences of the markers for hepatitis C, Human T-cell lymphotropic virus, Chagas disease and HBs-Ag were 0.15%, 0.09%, 0.13% and 0.18%, respectively. The blood bank of Governador Valadares had the highest percentage of positive anti-HBc donors (2.41%). With regard to human immunodeficiency virus, the blood bank of Além Paraíba had the lowest percentage of positive donors while the blood banks of Juiz de Fora and Betim had the highest percentages. The blood bank in the city of Montes Claros had the highest prevalence of the marker for Chagas disease (0.69%). CONCLUSIONS: Data on the profile of serological ineligibility by the blood banks of the Fundação Hemominas highlights the particularities of each region thereby contributing to measures for health surveillance and helping the blood donation network in its donor selection procedures aimed at improving blood transfusion safety. <![CDATA[A new index to discriminate between iron deficiency anemia and thalassemia trait]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300214&lng=en&nrm=iso&tlng=en ABSTRACT BACKGROUND: The most common microcytic and hypochromic anemias are iron deficiency anemia and thalassemia trait. Several indices to discriminate iron deficiency anemia from thalassemia trait have been proposed as simple diagnostic tools. However, some of the best discriminative indices use parameters in the formulas that are only measured in modern counters and are not always available in small laboratories. The development of an index with good diagnostic accuracy based only on parameters derived from the blood cell count obtained using simple counters would be useful in the clinical routine. Thus, the aim of this study was to develop and validate a discriminative index to differentiate iron deficiency anemia from thalassemia trait. METHODS: To develop and to validate the new formula, blood count data from 106 (thalassemia trait: 23 and iron deficiency: 83) and 185 patients (thalassemia trait: 30 and iron deficiency: 155) were used, respectively. Iron deficiency, ß-thalassemia trait and a-thalassemia trait were confirmed by gold standard tests (low serum ferritin for iron deficiency anemia, HbA2 &gt; 3.5% for ß-thalassemia trait and using molecular biology for the a-thalassemia trait). RESULTS: The sensitivity, specificity, efficiency, Youden's Index, area under receiver operating characteristic curve and Kappa coefficient of the new formula, called the Matos &amp; Carvalho Index were 99.3%, 76.7%, 95.7%, 76.0, 0.95 and 0.83, respectively. CONCLUSION: The performance of this index was excellent with the advantage of being solely dependent on the mean corpuscular hemoglobin concentration and red blood cell count obtained from simple automatic counters and thus may be of great value in underdeveloped and developing countries. <![CDATA[Perceptions of donors and recipients regarding blood donation]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300220&lng=en&nrm=iso&tlng=en ABSTRACT OBJECTIVE: The aim of this study was to identify the perceptions of blood donors and recipients regarding the act of donating blood. METHOD: This descriptive study with a survey design focuses on subjective and cultural aspects. Twenty donors and 20 recipients in the blood bank at the time of data collection participated in the study. Interviews were analyzed according to deductive thematic analysis. RESULTS: Two themes emerged - perceptions of donors and perceptions of recipients. Both groups saw the act of donating blood as something positive, though donors associated their reports with the experiences of people close to them who needed blood transfusions, while the recipients associated donations with the maintenance of their lives as, for them, a blood transfusion was a necessary medical treatment. CONCLUSIONS: Perceptions regarding blood donations are culturally constructed, as the participants associated knowledge acquired in the social world with moral issues and their life experiences. Hence, in addition to helping others, these individuals feel socially and morally rewarded. <![CDATA[Assessment of blood sample stability for complete blood count using the Sysmex XN-9000 and Mindray BC-6800 analyzers]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300225&lng=en&nrm=iso&tlng=en ABSTRACT BACKGROUND: Different hematological analyzers have different analytical performances that are often reflected in the criteria for sample stability of the complete blood count. This study aimed to assess the stability of several hematological parameters using the XN-9000 Sysmex and BC-6800 Mindray analyzers. METHODS: The impact of storage at room temperature and 4 °C was evaluated after 2, 4, 6, 8, 24, 36 and 48 h using ten normal and 40 abnormal blood samples. The variation from the baseline measurement was evaluated by the Steel-Dwass-Critchlow-Fligner test and by Bland-Altman plots, using quality specifications and critical difference as the total allowable variation. RESULTS: Red blood cells and reticulocyte parameters (i.e. hematocrit, mean corpuscular volume, mean corpuscular hemoglobin concentration, red blood cell distribution width, immature reticulocyte fractions, low-fluorescence reticulocytes, middle-fluorescence reticulocytes, high fluorescence mononuclear cells) showed less stability compared to leukocyte and platelet parameters (except for monocyte count and mean platelet volume). The bias for hematocrit, mean corpuscular volume, mean corpuscular hemoglobin concentration and red blood cell distribution width coefficient of variation was higher than the critical difference after 8 h using both analyzers. CONCLUSION: Blood samples measured with both analyzers do not show analytically significant changes in up to 2 h of storage at room temperature and 4 °C. However, the maximum time for analysis can be extended for up to 8 h when the bias is compared to the critical difference. <![CDATA[Clinical, hematological and genetic data of a cohort of children with hemoglobin SD]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300240&lng=en&nrm=iso&tlng=en ABSTRACT INTRODUCTION: The hemoglobin FSD is very uncommon in newborn screening programs for sickle cell disease. In the program of Minas Gerais, Brazil, the clinical course of children with hemoglobin SD was observed to be heterogeneous. The objective of this study was to estimate the incidence (1999-2012) and to describe the natural history of a cohort of newborns with hemoglobin SD. METHODS: Isoelectric focusing was the primary method used in newborn screening. Polymerase chain reaction-restriction fragment length polymorphism and gene sequencing were used to identify mutant alleles and for haplotyping. Gap-polymerase chain reaction was used to detect alpha-thalassemia. RESULTS: Eleven cases of hemoglobin S/D-Punjab and eight of Hb S-Korle Bu were detected. Other variants with hemoglobin D mobility were not identified. All hemoglobin D-Punjab and hemoglobin Korle Bu alleles were associated with haplotype I. Among the children with hemoglobin S/D-Punjab, there were four with the ßS CAR haplotype, six with the Benin haplotype, and one atypical. Results of laboratory tests for hemoglobin S/D-Punjab and hemoglobin S-Korle Bu were: hemoglobin 8.0 and 12.3 g/dL (p-value &lt;0.001), leukocyte count 13.9 × 109/L and 10.5 × 109/L (p-value = 0.003), reticulocytes 7.5% and 1.0% (p-value &lt;0.001), hemoglobin F concentration 16.1% and 6.9% (p-value = 0.001) and oxygen saturation 91.9% and 97% (p-value = 0.002), respectively. Only hemoglobin S/D-Punjab children had acute pain crises and needed blood transfusions or hydroxyurea. Those with the Benin ßS haplotype had higher total hemoglobin and hemoglobin F concentrations compared to the CAR haplotype. Transcranial Doppler was normal in all children. CONCLUSION: The clinical course and blood cell counts of children with hemoglobin S/D-Punjab were very similar to those of hemoglobin SS children. In contrast, children with hemoglobin S-Korle Bu had clinical course and blood cell counts like children with the sickle cell trait. <![CDATA[Age-adjusted international prognostic index is a predictor of survival in gastric diffuse B-cell non-Hodgkin lymphoma patients]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300247&lng=en&nrm=iso&tlng=en ABSTRACT BACKGROUND: The clinical course of gastric lymphoma is heterogeneous and clinical symptoms and some factors have been related to prognosis. OBJECTIVE: The present study aims to identify prognostic factors in gastric diffuse B-cell non-Hodgkin lymphoma diagnosed and treated in different countries. METHODS: A consecutive series of gastric diffuse B-cell non-Hodgkin lymphoma patients diagnosed and treated in Brazil, Portugal and Italy, between February 2008 and December 2014 was evaluated. RESULTS: Of 104 patients, 57 were female and the median age was 69 years (range: 28-88). The distribution of the age-adjusted international prognostic index was 12/95 (13%) high risk, 20/95 (21%) high-intermediate risk and 63/95 (66%) low/low-intermediate risk. Symptoms included abdominal pain (63/74), weight loss (57/73), dysphagia (37/72) and nausea/vomiting (37/72). Bulky disease was found in 24% of the cases, anemia in 33 of 76 patients and bleeding in 22 of 72 patients. The median follow-up time was 25 months (range: 1-77 months), with 1- and 5-year survival rates of 79% and 76%, respectively. The multivariate Cox Regression identified the age-adjusted international prognostic index as a predictor of death (hazard risk: 3.62; 95% confidence interval: 2.21-5.93; p-value &lt;0.0001). CONCLUSIONS: This series identified the age-adjusted international prognostic index as predictive of mortality in patients treated with conventional immunochemotherapy. <![CDATA[Transfusion medicine in medical education: an analysis of curricular grids in Brazil and a review of the current literature]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300252&lng=en&nrm=iso&tlng=en ABSTRACT BACKGROUND: Blood transfusions are one of the most performed medical procedures in the world. Thus, as education in transfusion medicine is vital to medical care, it should aim to promote a responsible practice with the rational use of blood by doctors. This study aims to investigate the situation of the teaching of transfusion medicine in medical schools in Brazil. METHOD: The websites of the 249 Brazilian medical schools in operation in June 2015 were visited and the curricula of the medical courses were investigated in respect to the presence or absence of a transfusion medicine discipline. When available, the subject grids were analyzed to verify whether a description of content regarding transfusion medicine was given within other disciplines. RESULTS: Of the 249 medical school sites visited, information on the curriculum was obtained from 178. Of the medical schools that published their curriculum, 132 (74.1%) did not have disciplines of transfusion medicine or hematology and only seven (3.9%) had a discipline of transfusion medicine in the curricular grid. CONCLUSIONS: Education on transfusion medicine is of fundamental importance for safe and efficient transfusion practices. Deficiencies in medical knowledge of this subject have been found worldwide. The results of this study indicate a possible deficiency in teaching the basics of this specialty. Thus, additional prospective studies to assess the knowledge and practice of transfusion medicine in Brazilian medical schools are warranted, which could prompt a discussion on the importance of offering training in transfusion medicine to medical students. <![CDATA[Primary myelofibrosis: current therapeutic options]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300257&lng=en&nrm=iso&tlng=en ABSTRACT Primary myelofibrosis is a Philadelphia-negative myeloproliferative neoplasm characterized by clonal myeloid expansion, followed by progressive fibrous connective tissue deposition in the bone marrow, resulting in bone marrow failure. Clonal evolution can also occur, with an increased risk of transformation to acute myeloid leukemia. In addition, disabling constitutional symptoms secondary to the high circulating levels of proinflammatory cytokines and hepatosplenomegaly frequently impair quality of life. Herein the main current treatment options for primary myelofibrosis patients are discussed, contemplating disease-modifying therapeutics in addition to palliative measures, in an individualized patient-based approach. <![CDATA[Diminished expression of B antigen mimicking B3 phenotype in a patient with AML-M3: a rare case report]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300264&lng=en&nrm=iso&tlng=en ABSTRACT Primary myelofibrosis is a Philadelphia-negative myeloproliferative neoplasm characterized by clonal myeloid expansion, followed by progressive fibrous connective tissue deposition in the bone marrow, resulting in bone marrow failure. Clonal evolution can also occur, with an increased risk of transformation to acute myeloid leukemia. In addition, disabling constitutional symptoms secondary to the high circulating levels of proinflammatory cytokines and hepatosplenomegaly frequently impair quality of life. Herein the main current treatment options for primary myelofibrosis patients are discussed, contemplating disease-modifying therapeutics in addition to palliative measures, in an individualized patient-based approach. <![CDATA[Renal papillary necrosis in a patient with sickle cell disease]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300267&lng=en&nrm=iso&tlng=en ABSTRACT Primary myelofibrosis is a Philadelphia-negative myeloproliferative neoplasm characterized by clonal myeloid expansion, followed by progressive fibrous connective tissue deposition in the bone marrow, resulting in bone marrow failure. Clonal evolution can also occur, with an increased risk of transformation to acute myeloid leukemia. In addition, disabling constitutional symptoms secondary to the high circulating levels of proinflammatory cytokines and hepatosplenomegaly frequently impair quality of life. Herein the main current treatment options for primary myelofibrosis patients are discussed, contemplating disease-modifying therapeutics in addition to palliative measures, in an individualized patient-based approach. <![CDATA[Transient red cell aplasia in two brothers with sickle cell anemia and erythrovirus B19 infection]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300271&lng=en&nrm=iso&tlng=en ABSTRACT Primary myelofibrosis is a Philadelphia-negative myeloproliferative neoplasm characterized by clonal myeloid expansion, followed by progressive fibrous connective tissue deposition in the bone marrow, resulting in bone marrow failure. Clonal evolution can also occur, with an increased risk of transformation to acute myeloid leukemia. In addition, disabling constitutional symptoms secondary to the high circulating levels of proinflammatory cytokines and hepatosplenomegaly frequently impair quality of life. Herein the main current treatment options for primary myelofibrosis patients are discussed, contemplating disease-modifying therapeutics in addition to palliative measures, in an individualized patient-based approach. <![CDATA[Gingival swelling associated with hypoplasminogenemia]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842016000300274&lng=en&nrm=iso&tlng=en ABSTRACT Primary myelofibrosis is a Philadelphia-negative myeloproliferative neoplasm characterized by clonal myeloid expansion, followed by progressive fibrous connective tissue deposition in the bone marrow, resulting in bone marrow failure. Clonal evolution can also occur, with an increased risk of transformation to acute myeloid leukemia. In addition, disabling constitutional symptoms secondary to the high circulating levels of proinflammatory cytokines and hepatosplenomegaly frequently impair quality of life. Herein the main current treatment options for primary myelofibrosis patients are discussed, contemplating disease-modifying therapeutics in addition to palliative measures, in an individualized patient-based approach.