Scielo RSS <![CDATA[Revista Brasileira de Hematologia e Hemoterapia]]> http://www.scielo.br/rss.php?pid=1516-848420150003&lang=es vol. 37 num. 3 lang. es <![CDATA[SciELO Logo]]> http://www.scielo.br/img/en/fbpelogp.gif http://www.scielo.br <![CDATA[Fetal hemoglobin and hemolysis markers in sickle cell anemia]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300148&lng=es&nrm=iso&tlng=es <![CDATA[The compound state: Hb S/beta-thalassemia]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300150&lng=es&nrm=iso&tlng=es <![CDATA[Evaluation of the effectiveness of packed red blood cell irradiation by a linear accelerator]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300153&lng=es&nrm=iso&tlng=es Irradiation of blood components with ionizing radiation generated by a specific device is recommended to prevent transfusion-associated graft-versus-host disease. However, a lin- ear accelerator can also be used in the absence of such a device, which is the case of the blood bank facility studied herein. In order to evaluate the quality of the irradiated packed red blood cells, this study aimed to determine whether the procedure currently employed in the facility is effective in inhibiting the proliferation of T lymphocytes without damaging blood components. The proliferation of T lymphocytes, plasma potassium levels, and the degree of hemolysis were evaluated and compared to blood bags that received no irradiation. Packed red blood cell bags were irradiated at a dose of 25 Gy in a linear accelerator. For this purpose, a container was designed to hold the bags and to ensure even distribution of irradiation as evaluated by computed tomography and dose-volume histogram. Irradiation was observed to inhibit the proliferation of lymphocytes. The percentage of hemolysis in irradiated bags was slightly higher than in non-irradiated bags (p-value &gt;0.05), but it was always less than 0.4% of the red cell mass. Although potassium increased in both groups, it was more pronounced in irradiated red blood cells, especially after seven days of storage, with a linear increase over storage time. The findings showed that, at an appropriate dosage and under validated conditions, the irradiation of packed red blood cells in a linear accelerator is effective, inhibiting lymphocyte proliferation but without compromising the viability of the red cells. <![CDATA[Mobilization and collection of CD34+ cells for autologous transplantation of peripheral blood hematopoietic progenitor cells in children: analysis of two different granulocyte-colony stimulating factor doses]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300160&lng=es&nrm=iso&tlng=es Introduction: The use of peripheral hematopoietic progenitor cells (HPCs) is the cell choice in autologous transplantation. The classic dose of granulocyte-colony stimulating factor (G- CSF) for mobilization is a single daily dose of 10 µg/kg of patient body weight. There is a theory that higher doses of granulocyte-colony stimulating factor applied twice daily could increase the number of CD34+ cells collected in fewer leukapheresis procedures. Objective: The aim of this study was to compare a fractionated dose of 15 µg G-CSF/kg of body weight and the conventional dose of granulocyte-colony stimulating factor in respect to the number of leukapheresis procedures required to achieve a minimum collection of 3 × 106 CD34+ cells/kg body weight. Methods: Patients were divided into two groups: Group 10 - patients who received a single daily dose of 10 µg G-CSF/kg body weight and Group 15 - patients who received a fractioned dose of 15 µg G-CSF/kg body weight daily. The leukapheresis procedure was carried out in an automated cell separator. The autologous transplantation was carried out when a minimum number of 3 × 106 CD34+ cells/kg body weight was achieved. Results: Group 10 comprised 39 patients and Group 15 comprised 26 patients. A total of 146 apheresis procedures were performed: 110 (75.3%) for Group 10 and 36 (24.7%) for Group 15. For Group 10, a median of three (range: 1-7) leukapheresis procedures and a mean of 8.89 × 106 CD34+ cells/kg body weight (±9.59) were collected whereas for Group 15 the corresponding values were one (range: 1-3) and 5.29 × 106 cells/kg body weight (±4.95). A statistically significant difference was found in relation to the number of apheresis procedures (p-value &lt;0.0001). Conclusions: To collect a minimum target of 3 × 106 CD34+ cells/kg body weight, the administration of a fractionated dose of 15 µg G-CSF/kg body weight significantly decreased the number of leukapheresis procedures performed. <![CDATA[Pattern of hemolysis parameters and association with fetal hemoglobin in sickle cell anemia patients in steady state]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300167&lng=es&nrm=iso&tlng=es Objective: This study aimed to evaluate the influence of fetal hemoglobin (Hb F) on hemolysis biomarkers in sickle cell anemia patients. Methods: Fifty adult sickle cell anemia patients were included in the study. All patients were taking hydroxyurea for at least six months and were followed at the outpatient clinic of a hospital in Fortaleza, Ceará, Brazil. The control group consisted of 20 hemoglobin AA individuals. The reticulocyte count was performed by an automated methodology, lactate dehydrogenase and uric acid were measured by spectrophotometry and arginase I by enzyme-linked immunosorbent assay (ELISA). The presence of Hb S was detected by high-performance liquid chromatography. The level of significance was set for a p-value &lt;0.05. Results: A significant increase was observed in the reticulocyte count and lactate dehydrogenase, uric acid and arginase I levels in sickle cell anemia patients compared to the control group (p-value &lt;0.05). Patients having Hb F levels greater than 10% showed a significant decrease in the reticulocyte count, arginase I and lactate dehydrogenase. A significant decrease was observed in arginase I levels in patients taking hydroxyurea at a dose greater than 20 mg/kg/day. Conclusion: The results of this study show that sickle cell anemia patients have increases in the hemolysis biomarkers, lactate dehydrogenase, reticulocyte count, arginase I, uric acid and increases in Hb F can reduce the reticulocyte count and arginase I and lactate dehydrogenase levels. <![CDATA[Socioeconomic and demographic characteristics of sickle cell disease patients from a low-income region of northeastern Brazil]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300172&lng=es&nrm=iso&tlng=es Objective: To characterize the socioeconomic and demographic aspects of sickle cell disease patients from the state of Rio Grande do Norte (RN), Northeast Brazil, and their adherence to the recommended treatment. Methods: This cross-sectional descriptive study was performed at referral centers for the treatment of hematological diseases. One hundred and fifty-five unrelated individuals with sickle cell disease who went to these centers for outpatient visits were analyzed. All the patients, or their caregivers, were informed about the research procedures and objectives, and answered a standardized questionnaire. Results: The patients were predominantly younger than 12 years old, self-declared as mulatto, lived in small towns fairly distant from the referral center, and had low education and socioeconomic levels. Individuals who were ten or younger were diagnosed at an earlier age. Almost 50% of the patients were taking hydroxyurea, 91.4% reported having received pneumococcal/meningococcal vaccinations and 76.1% received penicillin as antibiotic prophylaxis. However, the majority of them reported having difficulties following the recommendations of the physicians, mainly in respect to attaining the prescribed medications and transportation to the referral centers. Conclusion: These individuals have a vulnerable socioeconomic situation that can lead to an aggravation of their general health and thus deserve special attention from the medical and psychosocial perspectives. Thus, it is necessary to improve public policies that provide Brazilian sickle cell disease patients with better access to medical treatment, living conditions, and integration into society. <![CDATA[Evaluation of hemoglobin performance in the assessment of iron stores in feto-maternal pairs in a high-risk population: receiver operating characteristic curve analysis]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300178&lng=es&nrm=iso&tlng=es Objective: By applying receiver operating characteristic curve analysis, the objective of this study was to see whether hemoglobin levels reflect body iron stores in a group of pregnant women at term who, by using serum ferritin as the reference test, had a high pre-test prob- ability of having iron deficiency anemia. Likewise, we evaluated the ability of hemoglobin and maternal serum ferritin levels to predict iron deficiency anemia in newborns. Methods: Hemoglobin and serum ferritin were measured in 187 pregnant women at term belonging to a group with a high pre-test probability of iron deficiency anemia and their newborns. Women with Hb &lt;11.0 g/dL and newborns with cord Hb &lt;13.0 g/dL were classified as anemic. A serum ferritin &lt;12.0 µg/L in women and a cord blood serum ferritin &lt;35.0 µg/L were considered to reflect empty iron stores. Receiver operating characteristic curve analysis was applied to select the cut-off points that better reflected iron stores. Results: The Hb cut-off point selected by receiver operating characteristic curve analysis in women was &lt;11.5 g/dL (sensitivity: 60.82, specificity: 53.33%, Youden Index: 0.450). Most of the newborns had normal Hb which precluded this analysis. Maternal Hb &lt;11.0 g/dL was the cut-off point that best reflected iron deficiency anemia in newborns (sensitivity: 55.88%, specificity: 57.24%, Youden Index: 0.217). The best cut-off point of maternal serum ferritin to reflect empty iron stores in newborns was &lt;6.0 µg/L (sensitivity: 76.47%, specificity: 31.58%, Youden Index: 0.200). Conclusion: Hemoglobin concentration performed poorly to detect iron deficiency anemia in women at term with high risk for iron deficiency and their newborns. <![CDATA[Outcomes in relapsed Hodgkin's lymphoma treated with autologous and allogeneic hematopoietic cell transplantation at the Pontificia Universidad Católica de Chile]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300184&lng=es&nrm=iso&tlng=es Introduction: Hodgkin's lymphoma is a highly curable disease. Autologous and reduced intensity allogeneic hematopoietic cell transplantations are alternatives to treat relapsed patients. Here, we report on the results of one service using these procedures. Methods: All patients who underwent transplantations in our institution between 1996 and 2014 were retrospectively studied and demographics, toxicities and survival rate were analyzed. Results: This study evaluated 24 autologous and five reduced intensity allogeneic transplantations: the median ages of the patients were 29 and 32 years, respectively. At the time of autologous transplantation, ten patients were in complete remission, nine had chemosensitive disease but were not in complete remission, three had refractory disease and the status of two is unknown. In the allogeneic group, two were in complete remission and three had chemosensitive disease. The 5-year overall survival after autologous transplantation was 42% (66% patients were in complete remission, 37% had chemosensitive disease with incom- plete remission and 0% had refractory disease) and 1-year overall survival after allogeneic transplantation was 80%. Transplant-related mortality was 0% in patients conditioned with the ifosfamide/carboplatin/etoposide (ICE), carmustine/etoposide/cyclophosphamide (BEC) and carmustine/etoposide/cytarabine/melphalan (BEAM) regimens, 37% in patients condi- tioned with busulfan-based regimens and 20% in allogeneic transplantations. Conclusions: Hematopoietic cell transplantation for relapsed Hodgkin's lymphoma is a potentially curative procedure especially in patients in complete remission at the time of autologous transplantations, and possibly after allogeneic transplantations. Further studies are necessary to clarify the role of allogeneic transplantations in the treatment of relapsed Hodgkin's lymphoma. <![CDATA[Resistance of dialyzed patients to erythropoietin]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300190&lng=es&nrm=iso&tlng=es Resistance to recombinant human erythropoietin is a common condition in dialyzed patients with chronic kidney disease and is associated with more hospitalizations, increased mortality and frequent blood transfusions. The main cause of hyporesponsiveness to recombinant human erythropoietin in these patients is iron deficiency. However, a high proportion of patients does not respond to treatment, even to the use of intravenous iron, which indicates the presence of other important causes of resistance. In addition to the iron deficiency, the most common causes of resistance include inflammation, infection, malnutrition, inadequate dialysis, and hyperparathyroidism, although other factors may be associated. In the presence of adequate iron stores, other causes should be investigated and treated appropriately. <![CDATA[Very mild forms of Hb S/beta+-thalassemia in Brazilian children]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300198&lng=es&nrm=iso&tlng=es Resistance to recombinant human erythropoietin is a common condition in dialyzed patients with chronic kidney disease and is associated with more hospitalizations, increased mortality and frequent blood transfusions. The main cause of hyporesponsiveness to recombinant human erythropoietin in these patients is iron deficiency. However, a high proportion of patients does not respond to treatment, even to the use of intravenous iron, which indicates the presence of other important causes of resistance. In addition to the iron deficiency, the most common causes of resistance include inflammation, infection, malnutrition, inadequate dialysis, and hyperparathyroidism, although other factors may be associated. In the presence of adequate iron stores, other causes should be investigated and treated appropriately. <![CDATA[Compound heterozygous state of β-thalassemia with IVS1-5 (G→C) mutation and Indian deletion-inversion Gγ(Aγδβ)<sup>0</sup>-thalassemia in eastern India]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300202&lng=es&nrm=iso&tlng=es Resistance to recombinant human erythropoietin is a common condition in dialyzed patients with chronic kidney disease and is associated with more hospitalizations, increased mortality and frequent blood transfusions. The main cause of hyporesponsiveness to recombinant human erythropoietin in these patients is iron deficiency. However, a high proportion of patients does not respond to treatment, even to the use of intravenous iron, which indicates the presence of other important causes of resistance. In addition to the iron deficiency, the most common causes of resistance include inflammation, infection, malnutrition, inadequate dialysis, and hyperparathyroidism, although other factors may be associated. In the presence of adequate iron stores, other causes should be investigated and treated appropriately. <![CDATA[Central retinal vein occlusion as first manifestation of relapse in acute lymphoblastic leukemia]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300207&lng=es&nrm=iso&tlng=es Resistance to recombinant human erythropoietin is a common condition in dialyzed patients with chronic kidney disease and is associated with more hospitalizations, increased mortality and frequent blood transfusions. The main cause of hyporesponsiveness to recombinant human erythropoietin in these patients is iron deficiency. However, a high proportion of patients does not respond to treatment, even to the use of intravenous iron, which indicates the presence of other important causes of resistance. In addition to the iron deficiency, the most common causes of resistance include inflammation, infection, malnutrition, inadequate dialysis, and hyperparathyroidism, although other factors may be associated. In the presence of adequate iron stores, other causes should be investigated and treated appropriately. <![CDATA[Somatic mutations of calreticulin in a Brazilian cohort of patients with myeloproliferative neoplasms]]> http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842015000300211&lng=es&nrm=iso&tlng=es Resistance to recombinant human erythropoietin is a common condition in dialyzed patients with chronic kidney disease and is associated with more hospitalizations, increased mortality and frequent blood transfusions. The main cause of hyporesponsiveness to recombinant human erythropoietin in these patients is iron deficiency. However, a high proportion of patients does not respond to treatment, even to the use of intravenous iron, which indicates the presence of other important causes of resistance. In addition to the iron deficiency, the most common causes of resistance include inflammation, infection, malnutrition, inadequate dialysis, and hyperparathyroidism, although other factors may be associated. In the presence of adequate iron stores, other causes should be investigated and treated appropriately.