Scielo RSS <![CDATA[Revista Brasileira de Hematologia e Hemoterapia]]> http://www.scielo.br/rss.php?pid=1516-848420120004&lang=en vol. 34 num. 4 lang. en <![CDATA[SciELO Logo]]> http://www.scielo.br/img/en/fbpelogp.gif http://www.scielo.br <![CDATA[<b>Anemia</b>: <b>winning elbow room in the field of hematology and hemotherapy</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400001&lng=en&nrm=iso&tlng=en <![CDATA[<b>Evaluation of respiratory conditions in early phase of hematopoietic stem cell transplantation</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400002&lng=en&nrm=iso&tlng=en <![CDATA[<b>Nitric oxide status in sickle cell anemia</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400003&lng=en&nrm=iso&tlng=en <![CDATA[<b>Comment on "The burden and quality of life of caregivers of sickle cell anemia patients taking hydroxyurea versus those not taking hydroxyurea"</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400004&lng=en&nrm=iso&tlng=en <![CDATA[<b>Immunity against hepatitis B and measles vaccination after chemotherapy for acute lymphoblastic leukaemia in children</b>: <b>revaccination policy</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400005&lng=en&nrm=iso&tlng=en <![CDATA[<b>Anti-<i>Trypanosoma cruzi </i>inconclusive results in blood donor screening</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400006&lng=en&nrm=iso&tlng=en <![CDATA[<b>Plasminogen and fibrinogen plasma levels in coronary artery disease</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400007&lng=en&nrm=iso&tlng=en <![CDATA[<b>Animal models of human nutritional diseases</b>: <b>a short overview</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400008&lng=en&nrm=iso&tlng=en <![CDATA[<b>Correlation of low levels of nitrite and high levels of fetal hemoglobin in patients with sickle cell disease at baseline</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400009&lng=en&nrm=iso&tlng=en BACKGROUND: Sickle cell disease is a hemoglobinopathy characterized by hemolytic anemia, increased susceptibility to infections and recurrent vaso-occlusive crises that reduces the quality of life of sufferers. OBJECTIVE: To evaluate the correlation of the levels of lactate dehydrogenase, malonaldehyde and nitrite to fetal hemoglobin in patients with sickle cell disease not under treatment with hydroxyurea in outpatients at a university hospital in Fortaleza, Ceará, Brazil. METHODS: Forty-four patients diagnosed with sickle cell disease were enrolled at baseline. Diagnosis was confirmed by evaluating the beta globin gene using polymerase chain reaction-restriction fragment length polymorphism. The concentration of fetal hemoglobin was obtained by high-performance liquid chromatography. Serum levels of nitrite, malonaldehyde and lactate dehydrogenase were measured by biochemical methods. RESULTS: Significantly higher levels of lactate dehydrogenase, nitrite and malonaldehyde were observed in patients with sickle cell disease compared to a control group. The study of the correlation between fetal hemoglobin levels and these variables showed a negative correlation with nitrite levels. No correlation was found between fetal hemoglobin and malonaldehyde or lactate dehydrogenase. When the study population was stratified according to fetal hemoglobin levels, a decrease in the levels of nitrite was observed with higher levels of fetal hemoglobin (p-value = 0.0415). CONCLUSION: The results show that, similar to fetal hemoglobin levels, the concentration of nitrite can predict the clinical course of the disease, but should not be used alone as a modulator of prognosis in patients with sickle cell disease. <![CDATA[<b>The burden and quality of life of caregivers of sickle cell anemia patients taking hydroxyurea versus those not taking hydroxyurea</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400010&lng=en&nrm=iso&tlng=en OBJECTIVE: To assess the burden and quality of life of caregivers of patients with sickle cell anemia taking hydroxyurea versus those of patients not taking hydroxyurea. METHODS: A cross-sectional study was performed of caregivers of outpatients with sickle cell anemia in two public hospitals in Campo Grande, MS, from January through June 2010. The World Health Organization Quality of Life-BREF Scale and the Caregiver Burden Scale were used. RESULTS: Of the 37 caregivers in this study, 81.1% were women, 73.0% were mothers, 59.5% were married, 54.1%were mulattos, 48.6% were housewives, 54.1% had family incomes of up to one minimum wage and 75.7% had onlycompleted elementary education. The mean duration of care provided (time after diagnosis) was 16.08 ± 9.88 yearsand 89.2% reported that they provided 24-hour care. Regarding health, 27.0% of study participants reported having physical and 13.5% emotional problems. There were no significant relationships between these variables either with the different domains or the total score of the WHOQOL-BREF comparing caregivers of patients taking hydroxyurea versusthose of patients not taking hydroxyurea. There was a moderate negative linear correlation between the WHOQOL-BREF and the Caregiver Burden Scale scores (linear correlation test of Pearson: p-value = 0.003, r = -0.477). The burden of caregivers of patients who did not take hydroxyurea was significantly higher than those of patients who took the medication in terms of general tension, disappointment, environment and total score (student t-test: p-value < 0.05). CONCLUSION: In the perception of the caregiver, looking after sickle cell anemia patients represents a moderate negative burden. <![CDATA[<b>Antibody responses to Hepatitis B and measles-mumps-rubella vaccines in children who received chemotherapy for acute lymphoblastic leukemia</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400011&lng=en&nrm=iso&tlng=en OBJECTIVE: To evaluate viral vaccine antibody levels in children with acute lymphoblastic leukemia after chemotherapy and after vaccine booster doses. METHODS: Antibody levels against hepatitis B, rubella, measles and mumps vaccine antigens were evaluated in 33 children after completing chemotherapy (before and after vaccine booster doses) and the results were compared to the data of 33 healthy children matched for gender, age and social class. RESULTS: After chemotherapy, 75.9%, 67.9%, 59.3% and 51.7% of the patients showed low antibody titers that would be unlikely to protect against exposure to measles, rubella, hepatitis B and mumps, respectively. After receiving a vaccine booster dose for these antigens the patients had high antibody levels consistent with potential protection against measles, mumps and hepatitis B, but not against rubella. CONCLUSION: Extra doses of measles-mumps-rubella plus hepatitis B vaccines are recommended in acute lymphoblastic leukemia patients submitted to treatment after hematologic recovery. After this, viral vaccine antibody levels should be verified to define the individual's protective status. <![CDATA[<b>Association of HLA antigens and BCR-ABL transcripts in leukemia patients with the Philadelphia chromosome</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400012&lng=en&nrm=iso&tlng=en OBJECTIVE: This study aimed to verify the association between human leukocyte antigens and the bcr-abl fusion protein resulting from t(9;22)(q34;q11) in chronic leukemia myeloid and acute lymphoblastic leukemia patients. METHODS: Forty-seven bcr-abl positive individuals were evaluated. Typing was performed bymicrolymphocytotoxicity and molecular biological methods (human leukocyte antigens Class I and Class II). A control group was obtained from the data of potential bone marrow donors registered in the Brazilian Bone Marrow Donor Registry (REDOME). RESULTS: Positive associations with HLA-A25 and HLA-B18 were found for the b2a2 transcript, as well as a tendency towards a positive association with HLA-B40 and a negative association with HLA-A68. The b3a2 transcript showed positive associations with HLA-B40 and HLA-DRB1*3. CONCLUSION: The negative association between human leukocyte antigens and the BCR-ABL transcript suggests that binding and presentation of peptides derived from the chimeric protein are effective to increase a cytotoxic T lymphocyte response appropriate for the destruction of leukemic cells. <![CDATA[<b>Leukocyte, red blood cell and morphological adaptation to moderate physical training in rats undernourished in the neonatal period</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400013&lng=en&nrm=iso&tlng=en OBJECTIVE: To analyze the impact of moderate physical exercise on the total and differential leukocyte counts and red blood cell count of 36 sixty-day-old adult male Wistar rats subjected to early malnourishment. METHODS: The rats were divided in nourished (N - casein 17%) and malnourished groups (M - casein 8%) and thesegroups were then subdivided in trained (T) untrained (U) creating four groups NT, NU, MT and MU. The NT and MTgroups were submitted to moderate physical exercise using a treadmill (60 min/day, 5 days/week for 8 weeks). Onthe 1st day, before the training started T0 and 24 hours after the last training day of the week (T1 until T8), a 1 mLaliquot of blood was collected from the animals' tails for analysis. The total leukocyte count was evaluated in a cellcounter with an electronic microscope. The cyanmethemoglobin technique was used to measure the hemoglobin level. The hematocrit values were determined as a percentage using the micro-hematocrit technique with a microcapillaryreader and a cell counter was used to determine the red blood cell count. The t-test was used for statistical analysis and a p-value < 0.05 was considered significant. Data are expressed as means ± standard deviation. RESULTS: There was a significant difference in the total leukocyte count between the NT (9.1 ± 0.1) and MT groups (8.0 ± 0.1) from T1 and in neutrophils between the NT (22.1 ± 0.6) and MT groups (24.6 ± 1.8) from T7 (p < 0.05). There was no statistical significance in the hemoglobin, hematocrit and red blood cell count from T1. CONCLUSIONS: According to the results of this study, moderate physical exercise seems to have induced physiologic adaptation in adult rats from T1. <![CDATA[<b>Performance of six diagnostic tests to screen for Chagas disease in blood banks and prevalence of<i> Trypanosoma cruzi</i> infection among donors with inconclusive serology screening based on the analysis of epidemiological variables</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400014&lng=en&nrm=iso&tlng=en OBJECTIVE: The frequent occurrence of inconclusive serology in blood banks and the absence of a gold standard test for Chagas'disease led us to examine the efficacy of the blood culture test and five commercial tests (ELISA, IIF, HAI, c-ELISA, rec-ELISA) used in screening blood donors for Chagas disease, as well as to investigate the prevalence of Trypanosoma cruzi infection among donors with inconclusive serology screening in respect to some epidemiological variables. METHODS: To obtain estimates of interest we considered a Bayesian latent class model with inclusion of covariates from the logit link. RESULTS: A better performance was observed with some categories of epidemiological variables. In addition, all pairs of tests (excluding the blood culture test) presented as good alternatives for both screening (sensitivity > 99.96% in parallel testing) and for confirmation (specificity > 99.93% in serial testing) of Chagas disease. The prevalence of 13.30% observed in the stratum of donors with inconclusive serology, means that probably most of these are non-reactive serology. In addition, depending on the level of specific epidemiological variables, the absence of infection can be predicted with a probability of 100% in this group from the pairs of tests using parallel testing. CONCLUSION: The epidemiological variables can lead to improved test results and thus assist in the clarification of inconclusive serology screening results. Moreover, all combinations of pairs using the five commercial tests are good alternatives to confirm results. <![CDATA[<b>Plasminogen and fibrinogen plasma levels in coronary artery disease</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400015&lng=en&nrm=iso&tlng=en OBJECTIVE: The formation of thrombi at the site of atherosclerotic lesions plays a central role in atherothrombosis. Impaired fibrinolysis may exacerbate pre-existing coronary artery disease and potentiate its evolution. While the fibrinogen plasma level has been strongly associated with the severity of coronary artery disease, its relevance in the evaluation of plasminogen in coronary artery disease patients remains unclear. This study evaluated fibrinogen and plasminogen levels in subjects with coronary artery disease as diagnosed by angiography. METHODS: This is a cross-sectional study. Blood samples obtained from 17 subjects with angiographically normal coronary arteries (controls), 12 with mild/moderate atheromatosis and 28 with severe atheromatosis were evaluated. Plasma plasminogen and fibrinogen levels were measured by chromogenic and coagulometric methods, respectively. RESULTS: Fibrinogen levels were significantly higher in the severe atheromatosis group compared to the other groups(p-value < 0.0001). A significant positive correlation was observed between the severity of coronary artery diseaseand increasing fibrinogen levels (r = 0.50; p-value < 0.0001) and between fibrinogen and plasminogen levels (r =0.46; p-value < 0.0001). There were no significant differences in the plasminogen levels between groups. CONCLUSION: Plasma fibrinogen, but not plasminogen levels were higher in patients with coronary artery disease compared to angiographically normal subjects. The plasma fibrinogen levels also appear to be associated with the severity of the disease. The results of this study provide no evidence of a significant correlation between plasma plasminogen levels and the progress of coronary stenosis in the study population. <![CDATA[<b>Prothrombin complex concentrates in warfarin anticoagulation reversal</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400016&lng=en&nrm=iso&tlng=en OBJECTIVE: The formation of thrombi at the site of atherosclerotic lesions plays a central role in atherothrombosis. Impaired fibrinolysis may exacerbate pre-existing coronary artery disease and potentiate its evolution. While the fibrinogen plasma level has been strongly associated with the severity of coronary artery disease, its relevance in the evaluation of plasminogen in coronary artery disease patients remains unclear. This study evaluated fibrinogen and plasminogen levels in subjects with coronary artery disease as diagnosed by angiography. METHODS: This is a cross-sectional study. Blood samples obtained from 17 subjects with angiographically normal coronary arteries (controls), 12 with mild/moderate atheromatosis and 28 with severe atheromatosis were evaluated. Plasma plasminogen and fibrinogen levels were measured by chromogenic and coagulometric methods, respectively. RESULTS: Fibrinogen levels were significantly higher in the severe atheromatosis group compared to the other groups(p-value < 0.0001). A significant positive correlation was observed between the severity of coronary artery diseaseand increasing fibrinogen levels (r = 0.50; p-value < 0.0001) and between fibrinogen and plasminogen levels (r =0.46; p-value < 0.0001). There were no significant differences in the plasminogen levels between groups. CONCLUSION: Plasma fibrinogen, but not plasminogen levels were higher in patients with coronary artery disease compared to angiographically normal subjects. The plasma fibrinogen levels also appear to be associated with the severity of the disease. The results of this study provide no evidence of a significant correlation between plasma plasminogen levels and the progress of coronary stenosis in the study population. <![CDATA[<b>Heterozygosis for hemoglobin Porto Alegre identified by a combination of laboratory diagnostic methodologies</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400017&lng=en&nrm=iso&tlng=en OBJECTIVE: The formation of thrombi at the site of atherosclerotic lesions plays a central role in atherothrombosis. Impaired fibrinolysis may exacerbate pre-existing coronary artery disease and potentiate its evolution. While the fibrinogen plasma level has been strongly associated with the severity of coronary artery disease, its relevance in the evaluation of plasminogen in coronary artery disease patients remains unclear. This study evaluated fibrinogen and plasminogen levels in subjects with coronary artery disease as diagnosed by angiography. METHODS: This is a cross-sectional study. Blood samples obtained from 17 subjects with angiographically normal coronary arteries (controls), 12 with mild/moderate atheromatosis and 28 with severe atheromatosis were evaluated. Plasma plasminogen and fibrinogen levels were measured by chromogenic and coagulometric methods, respectively. RESULTS: Fibrinogen levels were significantly higher in the severe atheromatosis group compared to the other groups(p-value < 0.0001). A significant positive correlation was observed between the severity of coronary artery diseaseand increasing fibrinogen levels (r = 0.50; p-value < 0.0001) and between fibrinogen and plasminogen levels (r =0.46; p-value < 0.0001). There were no significant differences in the plasminogen levels between groups. CONCLUSION: Plasma fibrinogen, but not plasminogen levels were higher in patients with coronary artery disease compared to angiographically normal subjects. The plasma fibrinogen levels also appear to be associated with the severity of the disease. The results of this study provide no evidence of a significant correlation between plasma plasminogen levels and the progress of coronary stenosis in the study population. <![CDATA[<b>Abnormal transcranial Döppler ultrasonography in children with sickle cell disease</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400018&lng=en&nrm=iso&tlng=en BACKGROUND: Stroke is a potentially fatal complication of sickle cell disease in children between 2-16 years and transcranial Döppler has been recommended as a screening method in these cases. OBJECTIVE: The main goal of this study was to correlate transcranial Döppler results to complications related to stroke in sickle cell disease and baseline characteristics of the population. METHODS: This was an observational study of children and adolescents with ages between 2-16 years with sickle cell disease who were followed in three centers. RESULTS: From January 2008 to July 2009, 902 patients were enrolled in this study. The median age was 6.5 years (range: 1.8-15.8), 52.3% were male, 74.4% had hemoglobin SS; 221 (28.6%) had at least one complication associated with sickle cell disease. A total of 773 patients performed transcranial Döppler; in 91.2% this was a method of screening. Conditional or abnormal transcranial Döppler results were more common in patients with sickle cell disease complications versus those without complications (ODDS ratio = 3.18; 95% Confidence interval = 1.92-5.27). There was a significant difference in the frequency of conditional or abnormal transcranial Döppler results in patients with abnormal laboratory results compared to those without abnormalities (OR=4.03); 95% confidence interval = 2.30-7.06. CONCLUSIONS: Conditional or abnormal transcranial Döppler results were significantly more frequent in patients with complications of sickle cell disease confirming the increased risk of stroke in this subgroup of patients. This observation reinforces the recommendation of transcranial Döppler as a screening test for all patients with sickle cell disease with ages between 2 and 16 years. <![CDATA[<b>Non-HFE hemochromatosis</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400019&lng=en&nrm=iso&tlng=en Hereditary hemochromatosis (HH) is an autosomal recessive disorder classically related to HFE mutations. However, since 1996, it is known that HFE mutations explain about 80% of HH cases, with the remaining around 20% denominated non-HFE hemochromatosis. Nowadays, four main genes are implicated in the pathophysiology of clinical syndromes classified as non-HFE hemochromatosis: hemojuvelin (HJV, type 2Ajuvenile HH), hepcidin (HAMP, type 2B juvenile HH), transferrin receptor 2 (TFR2, type 3 HH) and ferroportin (SLC40A1, type 4 HH). The aim of this review is to explore molecular, clinical and management aspects of non-HFE hemochromatosis. <![CDATA[<b>Comet assay in myelodysplastic syndromes</b>]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842012000400020&lng=en&nrm=iso&tlng=en Hereditary hemochromatosis (HH) is an autosomal recessive disorder classically related to HFE mutations. However, since 1996, it is known that HFE mutations explain about 80% of HH cases, with the remaining around 20% denominated non-HFE hemochromatosis. Nowadays, four main genes are implicated in the pathophysiology of clinical syndromes classified as non-HFE hemochromatosis: hemojuvelin (HJV, type 2Ajuvenile HH), hepcidin (HAMP, type 2B juvenile HH), transferrin receptor 2 (TFR2, type 3 HH) and ferroportin (SLC40A1, type 4 HH). The aim of this review is to explore molecular, clinical and management aspects of non-HFE hemochromatosis.