Scielo RSS <![CDATA[Revista Brasileira de Hematologia e Hemoterapia]]> http://www.scielo.br/rss.php?pid=1516-848420140003&lang=pt vol. 36 num. 3 lang. pt <![CDATA[SciELO Logo]]> http://www.scielo.br/img/en/fbpelogp.gif http://www.scielo.br <![CDATA[Five years of implementation of guidelines in hematology and transfusion medicine in Brazil]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300165&lng=pt&nrm=iso&tlng=pt <![CDATA[Shed some (sun)light on vitamin D deficiency]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300167&lng=pt&nrm=iso&tlng=pt <![CDATA[Comments on: frequency of alleles and haplotypes of the human leukocyte antigen system in Bauru, São Paulo]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300169&lng=pt&nrm=iso&tlng=pt <![CDATA[Spirituality, religiousness and health: implications for the field of hematology]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300171&lng=pt&nrm=iso&tlng=pt <![CDATA[Utility of the p53 mutant protein in patients with low-risk myelodysplastic syndrome]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300173&lng=pt&nrm=iso&tlng=pt <![CDATA[Scientific comment on tumor suppressor p53 protein expression: prognostic significance in patients with low-risk myelodysplastic syndrome]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300175&lng=pt&nrm=iso&tlng=pt <![CDATA[Comment on: Homocysteine and vitamin B <sub>12</sub> status and iron deficiency anemia in female university students from Gaza, Palestine]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300178&lng=pt&nrm=iso&tlng=pt <![CDATA[Normal lymphocyte immunophenotype in an elderly population]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300180&lng=pt&nrm=iso&tlng=pt OBJECTIVE: The aim of this work was to evaluate the lymphocyte immunophenotype in an elderly population. METHODS: This study enrolled 35 over 60-year-old volunteers and a control group composed of 35 young adults. The study included elderly without diseases that might affect the functioning of the immune system. These individuals were consulted by doctors and after a physical examination, laboratory tests were performed using a Beckman Coulter (r) flow cytometer. The GraphPad Prism computer program was employed for statistical analysis with the level of significance being set for p-values &lt;0.05. RESULTS: There is a statistically significant reduction in the number of lymphocytes (CD8 +, CD2 + and CD3 + cells) in the elderly compared to young adults. These low rates are explained by changes attributed to aging and may be partly responsible for the reduction in the cellular immune response, lower proliferative activity and the low cytotoxicity of lymphocytes. CONCLUSION: These parameters showed greater impairment of adaptive immunity in the elderly population and can therefore explain the greater fragility of the aged body to developing diseases. <![CDATA[Association between religiousness and blood donation among Brazilian postgraduate students from health-related areas]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300184&lng=pt&nrm=iso&tlng=pt OBJECTIVE: The aim of this study was to examine the association between religiousness and blood donation among postgraduate students. METHODS: The Portuguese-language version of the Duke University Religion Index was administered to a sample of 226 Brazilian students with ages ranging from 22 to 55 years. All study participants had completed undergraduate courses in health-related areas. RESULTS: In the present study, 23.5% of the students were regular donors. Organizational religiousness was found to be associated with attitudes related to blood donation. This study also shows evidence that regular blood donors have a higher intrinsic religiousness than subjects who donate only once and do not return. CONCLUSION: This study shows that the attitudes concerning blood donation may have some association with religiosity. <![CDATA[Risk factors associated with the occurrence of adverse events in plateletpheresis donation]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300191&lng=pt&nrm=iso&tlng=pt OBJECTIVE: To recognize the profile of platelet donors and the profile of the plateletpheresis session as well as to investigate the main adverse events of platelet donation using plateletpheresis and associated risk factors. METHODS: This retrospective, cross-sectional and analytical study was performed with a quantitative approach by analyzing 316 donation files from February 2010 to December 2011. The IBM SPSS Statistics program was used for data processing and analysis. The chi-square test was used to verify whether there was an association between factors related to the procedure and the donor, and the adverse events that occurred. RESULTS: The mean age of platelet donors was 40 years old (standard deviation = 8.9), with the prevalent age group being between 40 and 49 years old; the prevalent blood type was O positive (53.8%), the mean duration of the procedure was 73 min and the mean amount of anticoagulant used was 360 mL. The association between procedure duration and the volume of anticoagulant was inverse and statistically significant; the longer the procedure and the greater the volume of anticoagulant used, the less adverse reactions occurred. CONCLUSION: The low incidence of adverse events indicates that the procedure is well tolerated by donors. Obtaining data regarding the incidence of adverse events is a way of promoting a dynamic review of medical and nursing teams to improve the safety and comfort of the donor. <![CDATA[Tumor suppressor p53 protein expression: prognostic significance in patients with low-risk myelodysplastic syndrome]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300196&lng=pt&nrm=iso&tlng=pt BACKGROUND: At the time of diagnosis, more than 50% of patients with myelodysplastic syndrome have a normal karyotype and are classified as having a favorable prognosis. However, these patients often show very variable clinical outcomes. Furthermore, current diagnostic tools lack the ability to look at genetic factors beyond karyotyping in order to determine the cause of this variability. OBJECTIVE: To evaluate the impact of p53 protein expression at diagnosis in patients with low-risk myelodysplastic syndrome. METHODS: This study enrolled 38 patients diagnosed with low-risk myelodysplastic syndrome. Clinical data were collected by reviewing medical records, and immunohistochemical p53 staining was performed on bone marrow biopsies. RESULTS: Of the 38 participants, 13 (34.21%) showed p53 expression in their bone marrow. At diagnosis, this group of patients also presented clinical features characteristic of a poor prognosis more often than patients who did not express p53. Furthermore, patients expressing p53 had a shorter median survival time compared to those without p53 expression. CONCLUSION: This study shows that the expression of p53 at diagnosis is a useful indicator of distinct clinical characteristics and laboratory profiles found in low-risk myelodysplastic syndrome patients. These data indicate that the immunohistochemical analysis of p53 may be a prognostic tool for myelodysplastic syndrome and should be used as an auxiliary test to help determine the best therapeutic choice. <![CDATA[Genetic evaluation of mesenchymal stem cells by G-banded karyotyping in a Cell Technology Center]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300202&lng=pt&nrm=iso&tlng=pt OBJECTIVE: To present the initial results of first three years of implementation of a genetic evaluation test for bone marrow-derived mesenchymal stem cells in a Cell Technology Center. METHODS: A retrospective study was carried out of 21 candidates for cell therapy. After the isolation of bone marrow mononuclear cells by density gradient, mesenchymal stem cells were cultivated and expanded at least until the second passage. Cytogenetic analyses were performed before and after cell expansion (62 samples) using G-banded karyotyping. RESULTS: All the samples analyzed, before and after cell expansion, had normal karyotypes, showing no clonal chromosomal changes. Signs of chromosomal instability were observed in 11 out of 21 patients (52%). From a total of 910 analyzed metaphases, five chromatid gaps, six chromatid breaks and 14 tetraploid cells were detected giving as total of 25 metaphases with chromosome damage (2.75%). CONCLUSION: The absence of clonal chromosomal aberrations in our results for G-banded karyotyping shows the maintenance of chromosomal stability of bone marrow-derived mesenchymal stem cells until the second passage; however, signs of chromosomal instability such as chromatid gaps, chromosome breaks and tetraploidy indicate that the long-term cultivation of these cells can provide an intermediate step for tumorigenesis. <![CDATA[Homocysteine and vitamin B <sub>12</sub> status and iron deficiency anemia in female university students from Gaza Strip, Palestine]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300208&lng=pt&nrm=iso&tlng=pt OBJECTIVE: Nutritional deficiencies are very significant to the overall health of humans at all ages and for both genders, yet in infants, children and women of childbearing age these deficiencies can seriously affect growth and development. The present work is aimed to assess homocysteine and vitamin B12 status in females with iron deficiency anemia from the Gaza Strip. METHODS: Venous blood samples were randomly collected from 240 female university students (18-22 years old) and parameters of the complete blood count, serum ferritin, homocysteine and vitamin B12 were measured. Statistical analysis included the t-test and analysis of variance (ANOVA) using the IBM SPSS software (version 18). Statistical significance was set for p-values &lt;0.05. RESULTS: The results revealed that 20.4% of the students have iron deficiency anemia. The mean serum vitamin B12 level in females with iron deficiency anemia (212.9 ± 62.8 pg/mL) was significantly lower than in normal controls (286.9 ± 57.1 pg/mL) and subjects with microcytic anemia and normal ferritin (256.7 ± 71.1 pg/mL). Significantly higher serum homocysteine levels were reported in the iron deficiency anemia group (27.0 ± 4.6 µmol/L) compared to normal controls (15.5 ± 2.9 µmol/L) and in subjects with microcytic anemia and normal ferritin (18.1 ± 2.7 µmol/L). Statistically significant negative correlations were reported for serum homocysteine with serum ferritin, vitamin B12, hemoglobin, and hematocrit levels. CONCLUSION: Important associations were found between serum homocysteine and markers of iron deficiency. Monitoring homocysteine levels might be essential to understand the development of different clinical conditions including anemia. It seems necessary to conduct prospective trials to determine whether treating anemia ameliorates homocysteine levels. <![CDATA[Murine leukemia virus-derived retroviral vector has differential integration patterns in human cell lines used to produce recombinant factor VIII]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300213&lng=pt&nrm=iso&tlng=pt OBJECTIVE: Nowadays recombinant factor VIII is produced in murine cells including in Chinese hamster ovary (CHO) and baby hamster kidney cells (BHK). Previous studies, using the murine leukemia virus-derived retroviral vector pMFG-FVIII-P140K, modified two recombinant human cell lines, HepG2 and Hek293 to produce recombinant factor VIII. In order to characterize these cells, the present study aimed to analyze the integration pattern of retroviral vector pMFG-FVIII-P140K. METHODS: This study used ligation-mediated polymerase chain reaction to locate the site of viral vector integration by sequencing polymerase chain reaction products. The sequences were compared to genomic databases to characterize respective clones. RESULTS: The retroviral vector presented different and non-random profiles of integration between cells lines. A preference of integration for chromosomes 19, 17 and 11 was observed for HepG2FVIIIdB/P140K and chromosome 9 for Hek293FVIIIdB/P140K. In genomic regions such as CpG islands and transcription factor binding sites, there was no difference in the integration profiles for both cell lines. Integration in intronic regions of encoding protein genes (RefSeq genes) was also observed in both cell lines. Twenty percent of integrations occurred at fragile sites in the genome of the HepG2 cell line and 17% in Hek293. CONCLUSION: The results suggest that the cell type can affect the profile of chromosomal integration of the retroviral vector used; these differences may interfere in the level of expression of recombinant proteins. <![CDATA[Are the review criteria for automated complete blood counts of the International Society of Laboratory Hematology suitable for all hematology laboratories?]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300219&lng=pt&nrm=iso&tlng=pt OBJECTIVE: to verify whether the review criteria for automated blood counts suggested by the International Consensus Group for Hematology Review of the International Society for Laboratory Hematology are suitable for the Hematology Laboratory of Hospital de Clinicas, Universidade Federal do Paraná. METHODS: initially, the review criteria of the International Society for Laboratory Hematology were adapted due to limitations in the Institution's electronic hospital records and interfacing systems. The adapted review criteria were tested using 1977 samples. After this first assessment, an additional 180 inpatient samples were analyzed to evaluate the screening criteria of the review criteria in conjunction with positive smear findings established by the institution. The performance of the review criteria was verified by determining false positive, false negative, true positive and true negative rates, sensitivity, specificity, positive predictive value, negative predictive value, microscopic review rate and efficiency. RESULTS: initial analysis showed false negatives = 6.73%, false positives = 23.27%, microscopic review rate = 46.03% and efficiency = 70.0%. An evaluation of the screening criteria adapted from the review criteria together with the positive smear findings of the institution showed false negatives = 15.5%, false positives = 10.5%, microscopic review rate = 37.3% and efficiency = 73.8%. In both situations the safety limit (false negative &lt;5%) recommended by the review criteria was exceeded. CONCLUSIONS: the review criteria adapted from the International Society for Laboratory Hematology are neither suitable nor safe for use in the hematology laboratory of the Hospital de Clinicas. This implies a need to develop and validate institution-specific review criteria in order to decrease false negative results to an acceptable and safe rate for patients. <![CDATA[Systemic mastocytosis - a diagnostic challenge]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300226&lng=pt&nrm=iso&tlng=pt Mastocytosis refers to a group of disorders characterized by the infiltration of clonally derived mast cells to the skin or extracutaneous tissues resulting in a heterogeneous clinical picture. It is a rare hematologic disorder in all its forms. The exact incidence is unknown; it affects patients of any age and males and females equally. Its molecular pathogenesis is incompletely understood. The clinical features of mastocytosis result from both chronic and episodic mast cell mediator release, signs and symptoms arising from diffuse or focal tissue infiltration, and, occasionally, the presence of an associated non-mast cell clonal hematologic disease. The histopathologic analysis is essential for definitive diagnosis but there is no curative treatment. The authors report a clinical case of a 72-year-old woman with no history of allergies, with bicytopenia, weight loss, and diffuse axial osteolytic lesions. This is a rare clinical case of aggressive systemic mastocytosis for which palliative treatment can improve survival and quality of life. A brief review of the literature about this pathology is also included. <![CDATA[De novo alpha 2 hemoglobin gene (HBA2) mutation in a child with hemoglobin M Iwate and symptomatic methemoglobinemia since birth]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300230&lng=pt&nrm=iso&tlng=pt Cyanosis in an apparently healthy newborn baby may be caused by hemoglobin variants associated with the formation of methemoglobin, collectively known as M hemoglobins. They should not be confused with genetic alterations in methemoglobin reductase enzyme systems of red cells since treatment and prognosis are completely different. A newborn male child was noted to be significantly cyanotic at birth and is the basis for this report. Hemoglobin isoelectric focusing, acid and alkaline gel electrophoresis, and HBA/HBB gene sequencing were performed for the child, both parents and a sister. The newborn child was treated with methylene blue in an intensive care unit fearing that he had a defective reductase system and exposure to oxidant drugs or toxins. Newborn hemoglobin screening with high performance liquid chromatography was abnormal on the 10th and 45th days but no conclusive diagnosis was reached. Cyanosis persisted up to four years of age with no other symptoms. Hemoglobin M Iwate [alpha2 87(F8) His&gt;Tyr, HBA2:c.262C&gt;T] was detected. It was not present in the child's presumed mother, father, sister, and brother. The analysis of 15 short tandem repeats in the trio demonstrated a de novo mutation occurrence (p-value &lt; 1 × 10 -8). The family was reassured that no further action was necessary and genetic counseling was provided. Methemoglobins should be considered for differential diagnosis of cyanosis in newborns even if no familial cases are detected. Except for cosmetic consequences, the clinical course of patients with hemoglobin M Iwate is unremarkable. <![CDATA[Thrombohemostatic disorders in the new emerging H7N9 influenza]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842014000300235&lng=pt&nrm=iso&tlng=pt Cyanosis in an apparently healthy newborn baby may be caused by hemoglobin variants associated with the formation of methemoglobin, collectively known as M hemoglobins. They should not be confused with genetic alterations in methemoglobin reductase enzyme systems of red cells since treatment and prognosis are completely different. A newborn male child was noted to be significantly cyanotic at birth and is the basis for this report. Hemoglobin isoelectric focusing, acid and alkaline gel electrophoresis, and HBA/HBB gene sequencing were performed for the child, both parents and a sister. The newborn child was treated with methylene blue in an intensive care unit fearing that he had a defective reductase system and exposure to oxidant drugs or toxins. Newborn hemoglobin screening with high performance liquid chromatography was abnormal on the 10th and 45th days but no conclusive diagnosis was reached. Cyanosis persisted up to four years of age with no other symptoms. Hemoglobin M Iwate [alpha2 87(F8) His&gt;Tyr, HBA2:c.262C&gt;T] was detected. It was not present in the child's presumed mother, father, sister, and brother. The analysis of 15 short tandem repeats in the trio demonstrated a de novo mutation occurrence (p-value &lt; 1 × 10 -8). The family was reassured that no further action was necessary and genetic counseling was provided. Methemoglobins should be considered for differential diagnosis of cyanosis in newborns even if no familial cases are detected. Except for cosmetic consequences, the clinical course of patients with hemoglobin M Iwate is unremarkable.