Scielo RSS <![CDATA[Revista Brasileira de Hematologia e Hemoterapia]]> http://www.scielo.br/rss.php?pid=1516-848420150001&lang=es vol. 37 num. 1 lang. es <![CDATA[SciELO Logo]]> http://www.scielo.br/img/en/fbpelogp.gif http://www.scielo.br <![CDATA[Many thanks to our eternal Editor of the Revista Brasileira de Hematologia e Hemoterapia, Professor Milton Artur Ruiz]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100001&lng=es&nrm=iso&tlng=es <![CDATA[Myeloid leukemia: are we getting better?]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100003&lng=es&nrm=iso&tlng=es <![CDATA[The invisibility of sickle cell disease in Brazil: lessons from a study in Maranhao]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100005&lng=es&nrm=iso&tlng=es <![CDATA[Trends in mortality of adult patients diagnosed with myeloid leukemia from 1994 to 2011 in southeastern Brazil]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100007&lng=es&nrm=iso&tlng=es Objective: To evaluate trends in mortality among adults with myeloid leukemia in the Vale do Paraíba, State of São Paulo. Methods: Data from the Brazilian National Health Service database DATASUS provided the number of deaths caused by myeloid leukemia and the number of inhabitants per year in the Regional Health Division XVII from 1994 to 2011. Registries were categorized according to gender into four age ranges (over 20 years, 20-49, 50-69 and over 70 years) for an estimation of the annual percent change for age-adjusted mortality rates. The percent changes were calculated using the Joinpoint regression analysis model. Results: Overall, a significant decline per year was demonstrated for the entire sample (over 20 years) across the 18-year period studied (annual percent change: −5.59%; 95% CI: −8.5 to −2.5% for males; p-value &lt; 0.05 and −7.02%; 95% CI −11.2 to −2.8% for females; p-value &lt; 0.05) with no significant difference between genders. In an analysis using two Joinpoints, significant drops were observed from 1994 to 2001 (annual percent change: −21.22%; 95% confidence interval: −27.9 to −13.9%; p-value &lt; 0.05) and from 1994 to 2003 (annual percent change: −12.86%; 95% confidence interval −22.2 to −2.5%; p-value &lt; 0.05) for men and women, respectively. The declining trends were greatest for patients aged over 70 years with the age-adjusted mortality rates in younger groups declining non-significantly except for males aged 50-69 years old. Conclusion: Our data suggest a significant decline per year in age-adjusted mortality rates of adult patients diagnosed with myeloid leukemia from 1994 to 2011 in the Vale do Paraíba, State of São Paulo. <![CDATA[Trends in mortality and hospital admissions of sickle cell disease patients before and after the newborn screening program in Maranhão, Brazil]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100012&lng=es&nrm=iso&tlng=es Objective: To assess the impact of the implementation of neonatal screening on hospitalization and death rates due to sickle cell disease in patients from the state of Maranhão, Brazil. Methods: A descriptive study was performed of all inpatients and deaths of patients with a diagnosis of sickle cell disease in Maranhão between 1999 and 2012. Data were collected from the Hospital Information System of the Brazilian National Health Service (SUS) and the Death Information System of the Ministry of Health. The implementation of newborn screening tests in Maranhão took place in 2005, and so the periods 1999-2005 (pre) and 2006-2012 (post) were analyzed for trend analysis using a multiple linear regression model. Fisher's exact test was used for the analysis of categorical variables and the Kruskal-Wallis test for continuous variables. Results: The rate of hospitalization increased from 0.315 (pre) to 1.832 (post), indicating 5.82 times more admissions (p-value = 0.04). The mortality rate increased from 0.115 to 0.216, that is 1.88 times higher, but this was not statistically significant (p-value = 0.586). The median age at admission dropped from 11.4 years to 8.7 years (p-value = 0.0002), whereas the median age at death increased from 10 years to 14 years (p-value = 0.665). Conclusion: The increases in the rates of hospitalization and death after the implementation of neonatal screening suggests that previously there was an underdiagnosis of sickle cell disease and that screening, along with other factors, increased "visibility" in the state of Maranhão. <![CDATA[Diversity of breakpoints of variant Philadelphia chromosomes in chronic myeloid leukemia in Brazilian patients]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100017&lng=es&nrm=iso&tlng=es Background: Chronic myeloid leukemia is a myeloproliferative disorder characterized by the Philadelphia chromosome or t(9;22)(q34.1;q11.2), resulting in the break-point cluster regionAbelson tyrosine kinase fusion gene, which encodes a constitutively active tyrosine kinase protein. The Philadelphia chromosome is detected by karyotyping in around 90% of chronic myeloid leukemia patients, but 5-10% may have variant types. Variant Philadelphia chromosomes are characterized by the involvement of another chromosome in addition to chromosome 9 or 22. It can be a simple type of variant when one other chromosome is involved, or complex, in which two or more chromosomes take part in the translocation. Few studies have reported the incidence of variant Philadelphia chromosomes or the breakpoints involved among Brazilian chronic myeloid leukemia patients. Objective: The aim of this report is to describe the diversity of the variant Philadelphia chromosomes found and highlight some interesting breakpoint candidates for further studies. Methods: the Cytogenetics Section Database was searched for all cases with diagnoses of chronic myeloid leukemia during a 12-year period and all the variant Philadelphia chromosomes were listed. Results: Fifty (5.17%) cases out of 1071 Philadelphia-positive chronic myeloid leukemia were variants. The most frequently involved chromosome was 17, followed by chromosomes: 1, 20, 6, 11, 2, 10, 12 and 15. Conclusion: Among all the breakpoints seen in this survey, six had previously been described: 11p15, 14q32, 15q11.2, 16p13.1, 17p13 and 17q21. The fact that some regions get more fre- quently involved in such rare rearrangements calls attention to possible predisposition that should be further studied. Nevertheless, the pathological implication of these variants remains unclear. <![CDATA[Acute myeloid leukemia: survival analysisof patients at a university hospital of Paraná]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100021&lng=es&nrm=iso&tlng=es Objective: The aim of this study was to analyze the prognostic factors correlated with survival of patients with acute myeloid leukemia at the Hospital de Clínicas, Universidade Federal do Paraná between 2003 and 2009, as well as to investigate the clinical and epidemiological profile. Methods: The overall survival and disease-free survival were statistically evaluated using the Kaplan-Meier method, the log-rank test and multivariate evaluation by Cox regression analysis. Results: The study population was predominantly younger than 60 years old (81,6%), had intermediate cytogenetic risk (40.8%), in first complete remission after induction chemotherapy (46.9%), with a white blood count at diagnosis of less than 30 × 109 /L (57.1%) and de novo acute myeloid leukemia (62.2%). Survival curves showed that better prognosis was related to age below 60 years (median:12,4 months; p-value = 0,2227; Odds Ratio = 0,6676), good pro- gnostic cytogenetic markers (median: 97.7 months; p-value = 0.0037; Odds Ratio = 0.4239) and white blood cell count at diagnosis of less than 30 × 109 /L (median survival: 23.6 months; p- value = 0.0001; Odds Ratio = 0.3651). Regarding the French-American-British subgroups, the median overall survival was 23.5 months for M0, M1 and M2, 97.7 months for M3 and 7.4 months for M4, M5, M6, and M7 (p-value = 0.0288). Conclusion: Prognostic factors strongly influenced patient survival, as well as guided treat- ment. Moreover, these factors were consistent with the available literature adjusted for the population in question. <![CDATA[Results of high-risk neutropenia therapy of hematology-oncology patients in a university hospital in Uruguay]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100028&lng=es&nrm=iso&tlng=es Background: Febrile neutropenia is an important cause of mortality and morbidity in hematology-oncology patients undergoing chemotherapy. The management of febrile neutropenia is typically algorithm-driven. The aim of this study was to assess the results of a standardized protocol for the treatment of febrile neutropenia. Methods: A retrospective cohort study (2011-2012) was conducted of patients with high-risk neutropenia in a hematology-oncology service. Results: Forty-four episodes of 17 patients with a median age of 48 years (range: 18-78 years) were included. The incidence of febrile neutropenia was 61.4%. The presence of febrile neutropenia was associated with both the duration and severity of neutropenia. Microbiological agents were isolated from different sources in 59.3% of the episodes with bacteremia iso- lated from blood being the most prevalent (81.3%). Multiple drug-resistant gram-negative bacilli were isolated in 62.5% of all microbiologically documented infections. Treatment of 63% of the episodes in which the initial treatment was piperacillin/tazobactam needed to be escalated to meropenem. The mortality rate due to febrile neutropenia episodes was 18.5%. Conclusion: The high rate of gram-negative bacilli resistant to piperacillin/tazobactam (frontline antibiotics in our protocol) and the early need to escalate to carbapenems raises the question as to whether it is necessary to change the current protocol. <![CDATA[Immunohistochemical evaluation of CD20 expression in patients with multiple myeloma]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100034&lng=es&nrm=iso&tlng=es Objective: CD20 expression was reported at different rates in patients with multiple myeloma. The importance of this B-cell antigen for plasma cells is still unknown. This study aimed to investigate CD20 expression of myeloma cells in bone marrow, and any relationship between the stage of disease, isotype and clinical features. Methods: Sixty-one patients who were admitted to the hematology clinic of the Adnan Menderes Medical School with the diagnosis of multiple myeloma according to the criteria of the "International Myeloma Working Group" were enrolled in this study. Age, gender, Durie-Salmon stage, history of autologous hematopoietic stem cell transplantation, and the distribution pattern and positivity of CD20 expression on multiple myeloma cells in bone marrow were evaluated. The Mann-Whitney U and chi-square tests were used for statistical analysis with a p-value &lt; 0.05 being accepted as statistically significant. Results: Thirty patients (48.9%) had positive scores for CD20 with the distribution pattern being most likely interstitial in 55.6% of the cases. There was no statistically significant difference between immunohistochemical positivity for CD20 expression on multiple myeloma cells, immunoglobulin type, and the stage of disease. Conclusion: The combination of immunohistochemical studies with flow cytometry may reveal the importance of CD20 positivity in patients with multiple myeloma more clearly. <![CDATA[Association between maternal and fetal factors and quality of cord blood as a source of stem cells]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100038&lng=es&nrm=iso&tlng=es Objectives: To comparatively analyze maternal and fetal factors and quality markers of blood samples in a public umbilical cord blood bank. Method: This is a cross-sectional descriptive study that revisited 458 records of donations from September 2009 to March 2013 at the Hemocentro de Santa Catarina. The means of markers were used to define cutoff points for the quality of cord blood. Results: Most donations came from women with ages between 18 and 29 years (62.8%), gestational age ≥ 40 weeks (55.2%), vaginal delivery (51.3%), primiparous (41.4%), and with male newborns (54.4%) weighing between 3000 and 3499 g (41.8%). The volume of the dona- tions ranged from 71.6 to 275.2 mL, the total nucleated cell count ranged from 4.77 × 108 to 31.0 × 108 cells and CD34+ cells ranged from 0.05 to 1.23%. There were statistically significant differences in the volume with respect to gestation age &gt; 38 weeks (p-value = 0.001), cesarean section (p-value &lt; 0.001) and birth weight &gt; 3500 g (p-value &lt; 0.001). The total nucleated cell count was positively affected by cesarean section (p-value = 0.022) and birth weight &gt; 3500 g (p-value &lt; 0.001). There was no statistically significant difference between the variables and the percentage of CD34+ cells. Conclusions: Delivery route and birth weight influence the volume of cord blood and the total nucleated cell count. Gestational age influences only the volume of cord blood. <![CDATA[Prevalence of anemia in a sample of elderly southeastern Brazilians]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100043&lng=es&nrm=iso&tlng=es Objective: To demonstrate the proportion of anemia and its association with demographic and clinical characteristics in a representative sample of elderly people from São José dos Campos, São Paulo. Methods: Demographic data and blood samples were collected from 398 over 65-year-old male and female individuals. Anemia was defined as hemoglobin concentration &lt;12 g/dL in women and &lt;13 g/dL in men. Anemic and non-anemic groups were compared using the chi-squared test and a multiple logistic regression model. Results: The prevalence of anemia was 18.6% (20.8% in men and 17.6% in women). The per- centages of anemia rose significantly across the age groups &gt;75-80, &gt;85-90 and &gt;90-95 years (p-value = 0.0251). There were no significant differences in gender, ethnic background, place of residence, years of schooling, income, comorbidities and use of medications. Accord- ing to gender, the mean hemoglobin concentration and mean corpuscular volume were 11.5 g/dL (range: 8.4-11.9 g/dL) and 90.7 fL (range: 63.0-111.7 fL) for women and 11.9 g/dL (range: 8.6-12.8 g/dL) and 92.1 fL (range: 59.8-100.1 fL) for men. The great majority of ane- mia cases were mild with less than 6% having hemoglobin concentrations below 10.9 g/dL. Mean corpuscular volume was lower than 80 fL in six cases (8%), between 80 and 100 fL in 65 cases (88%) and higher than 100 fL in three cases (4%). Conclusion: A total of 18.6% of elderly people from São José dos Campos had mild anemia with the majority being normocytic. The percentages of anemia rose as the age increased demonstrating an association between age and anemia. <![CDATA[Identifying the similarities and differences between single nucleotide polymorphism array (SNPa) analysis and karyotyping in acute myeloid leukemia and myelodysplastic syndromes]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100048&lng=es&nrm=iso&tlng=es Objective: To standardize the single nucleotide polymorphism array (SNPa) method in acute myeloid leukemia/myelodysplastic syndromes, and to identify the similarities and differ- ences between the results of this method and karyotyping. Methods: Twenty-two patients diagnosed with acute myeloid leukemia and three with myelodysplastic syndromes were studied. The G-banding karyotyping and single nucleotide polymorphism array analysis (CytoScan(r) HD) were performed using cells from bone marrow, DNA extracted from mononuclear cells from bone marrow and buccal cells (BC). Results: The mean age of the patients studied was 54 years old, and the median age was 55 years (range: 28-93). Twelve (48%) were male and 13 (52%) female. Ten patients showed abnormal karyotypes (40.0%), 11 normal (44.0%) and four had no mitosis (16.0%). Regarding the results of bone marrow single nucleotide polymorphism array analysis: 17 were abnor- mal (68.0%) and eight were normal (32.0%). Comparing the two methods, karyotyping identified a total of 17 alterations (8 deletions/losses, 7 trissomies/gains, and 2 translocations) and single nucleotide polymorphism array analysis identified a total of 42 alterations (17 losses, 16 gains and 9 copy-neutral loss of heterozygosity). Conclusion: It is possible to standardize single nucleotide polymorphism array analysis in acute myeloid leukemia/myelodysplastic syndromes and compare the results with the abnormalities detected by karyotyping. Single nucleotide polymorphism array analysis increased the detection rate of abnormalities compared to karyotyping and also identified a new set of abnormalities that deserve further investigation in future studies. <![CDATA[A case of chronic myeloid leukemia with the m-bcr (p190) molecular rearrangement identified during treatment]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100055&lng=es&nrm=iso&tlng=es Objective: To standardize the single nucleotide polymorphism array (SNPa) method in acute myeloid leukemia/myelodysplastic syndromes, and to identify the similarities and differ- ences between the results of this method and karyotyping. Methods: Twenty-two patients diagnosed with acute myeloid leukemia and three with myelodysplastic syndromes were studied. The G-banding karyotyping and single nucleotide polymorphism array analysis (CytoScan(r) HD) were performed using cells from bone marrow, DNA extracted from mononuclear cells from bone marrow and buccal cells (BC). Results: The mean age of the patients studied was 54 years old, and the median age was 55 years (range: 28-93). Twelve (48%) were male and 13 (52%) female. Ten patients showed abnormal karyotypes (40.0%), 11 normal (44.0%) and four had no mitosis (16.0%). Regarding the results of bone marrow single nucleotide polymorphism array analysis: 17 were abnor- mal (68.0%) and eight were normal (32.0%). Comparing the two methods, karyotyping identified a total of 17 alterations (8 deletions/losses, 7 trissomies/gains, and 2 translocations) and single nucleotide polymorphism array analysis identified a total of 42 alterations (17 losses, 16 gains and 9 copy-neutral loss of heterozygosity). Conclusion: It is possible to standardize single nucleotide polymorphism array analysis in acute myeloid leukemia/myelodysplastic syndromes and compare the results with the abnormalities detected by karyotyping. Single nucleotide polymorphism array analysis increased the detection rate of abnormalities compared to karyotyping and also identified a new set of abnormalities that deserve further investigation in future studies. <![CDATA[Teaching transfusion medicine: current situation and proposals for proper medical training]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100058&lng=es&nrm=iso&tlng=es The current curricula in medical schools and hospital residence worldwide lack exposure to blood transfusion medicine, and require the reformulation of academic programs. In many countries, training in blood transfusion is not currently offered to medical students or during residency. Clinical evidence indicates that blood transfusions occur more frequently than recommended, contributing to increased risk due to this procedure. Therefore, the rational use of blood and its components is essential, due to the frequent undesirable reactions, to the increasing demand of blood products and the cost of the process. Significant improvements in knowledge of and skills in transfusion medicine are needed by both students and residents. Improvements are needed in both background knowledge and the practical application of this knowledge to improve safety. Studies prove that hemovigilance has an impact on transfusion safety and helps to prevent the occurrence of transfusion-related adverse effects. To ensure that all these aspects of blood transfusion are being properly addressed, many countries have instituted hospital transfusion committees. From this perspective, the interventions performed during the formation of medical students and residents, even the simplest, have proven effective in the acquisition of knowledge and medical training, thereby leading to a reduction in inappropriate use of blood. Therefore, we would like to emphasize the importance of the exposure of medical students and residents to blood ser- vices and transfusion medicine in order for them to acquire adequate medical training, as well as to discuss some changes in the current medical curricula regarding transfusion medicine that we judge critical. <![CDATA[Motivating medical students to learn basic science concepts using chronic myeloid leukemia as an integration theme]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100063&lng=es&nrm=iso&tlng=es Objective: To report on the use of chronic myeloid leukemia as a theme of basic clinical integration for first year medical students to motivate and enable in-depth understanding of the basic sciences of the future physician. Methods: During the past thirteen years we have reviewed and updated the curriculum of the medical school of the Universidade Estadual de Campinas. The main objective of the new curriculum is to teach the students how to learn to learn. Since then, a case of chronic myeloid leukemia has been introduced to first year medical students and discussed in horizontal integration with all themes taught during a molecular and cell biology course. Cell structure and components, protein, chromosomes, gene organization, proliferation, cell cycle, apoptosis, signaling and so on are all themes approached during this course. At the end of every topic approached, the students prepare in advance the corresponding topic of clinical cases chosen randomly during the class, which are then presented by them. During the final class, a paper regarding mutations in the abl gene that cause resistance to tyrosine kinase inhibitors is discussed. After each class, three tests are solved in an interactive evaluation. Results: The course has been successful since its beginning, 13 years ago. Great motivation of those who participated in the course was observed. There were less than 20% absences in the classes. At least three (and as many as nine) students every year were interested in starting research training in the field of hematology. At the end of each class, an interactive evaluation was performed and more than 70% of the answers were correct in each evaluation. Moreover, for the final evaluation, the students summarized, in a written report, the molecular and therapeutic basis of chronic myeloid leukemia, with scores ranging from 0 to 10. Considering all 13 years, a median of 78% of the class scored above 5 (min 74%-max 85%), and a median of 67% scored above 7. Conclusion: Chronic myeloid leukemia is an excellent example of a disease that can be used for clinical basic integration as this disorder involves well known protein, cytogenetic and cell function abnormalities, has well-defined diagnostic strategies and a target oriented therapy. <![CDATA[Why and how validate criteria by manual smear review to improve laboratory productivity?]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100067&lng=es&nrm=iso&tlng=es Objective: To report on the use of chronic myeloid leukemia as a theme of basic clinical integration for first year medical students to motivate and enable in-depth understanding of the basic sciences of the future physician. Methods: During the past thirteen years we have reviewed and updated the curriculum of the medical school of the Universidade Estadual de Campinas. The main objective of the new curriculum is to teach the students how to learn to learn. Since then, a case of chronic myeloid leukemia has been introduced to first year medical students and discussed in horizontal integration with all themes taught during a molecular and cell biology course. Cell structure and components, protein, chromosomes, gene organization, proliferation, cell cycle, apoptosis, signaling and so on are all themes approached during this course. At the end of every topic approached, the students prepare in advance the corresponding topic of clinical cases chosen randomly during the class, which are then presented by them. During the final class, a paper regarding mutations in the abl gene that cause resistance to tyrosine kinase inhibitors is discussed. After each class, three tests are solved in an interactive evaluation. Results: The course has been successful since its beginning, 13 years ago. Great motivation of those who participated in the course was observed. There were less than 20% absences in the classes. At least three (and as many as nine) students every year were interested in starting research training in the field of hematology. At the end of each class, an interactive evaluation was performed and more than 70% of the answers were correct in each evaluation. Moreover, for the final evaluation, the students summarized, in a written report, the molecular and therapeutic basis of chronic myeloid leukemia, with scores ranging from 0 to 10. Considering all 13 years, a median of 78% of the class scored above 5 (min 74%-max 85%), and a median of 67% scored above 7. Conclusion: Chronic myeloid leukemia is an excellent example of a disease that can be used for clinical basic integration as this disorder involves well known protein, cytogenetic and cell function abnormalities, has well-defined diagnostic strategies and a target oriented therapy. <![CDATA[To follow or not to follow the recommendations regarding microscopic analysis of the Clinical and Laboratory Standards Institute H20-A2 to validate the criteria for blood smear review?]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000100069&lng=es&nrm=iso&tlng=es Objective: To report on the use of chronic myeloid leukemia as a theme of basic clinical integration for first year medical students to motivate and enable in-depth understanding of the basic sciences of the future physician. Methods: During the past thirteen years we have reviewed and updated the curriculum of the medical school of the Universidade Estadual de Campinas. The main objective of the new curriculum is to teach the students how to learn to learn. Since then, a case of chronic myeloid leukemia has been introduced to first year medical students and discussed in horizontal integration with all themes taught during a molecular and cell biology course. Cell structure and components, protein, chromosomes, gene organization, proliferation, cell cycle, apoptosis, signaling and so on are all themes approached during this course. At the end of every topic approached, the students prepare in advance the corresponding topic of clinical cases chosen randomly during the class, which are then presented by them. During the final class, a paper regarding mutations in the abl gene that cause resistance to tyrosine kinase inhibitors is discussed. After each class, three tests are solved in an interactive evaluation. Results: The course has been successful since its beginning, 13 years ago. Great motivation of those who participated in the course was observed. There were less than 20% absences in the classes. At least three (and as many as nine) students every year were interested in starting research training in the field of hematology. At the end of each class, an interactive evaluation was performed and more than 70% of the answers were correct in each evaluation. Moreover, for the final evaluation, the students summarized, in a written report, the molecular and therapeutic basis of chronic myeloid leukemia, with scores ranging from 0 to 10. Considering all 13 years, a median of 78% of the class scored above 5 (min 74%-max 85%), and a median of 67% scored above 7. Conclusion: Chronic myeloid leukemia is an excellent example of a disease that can be used for clinical basic integration as this disorder involves well known protein, cytogenetic and cell function abnormalities, has well-defined diagnostic strategies and a target oriented therapy.