Showing papers in "Revista Brasileira De Hematologia E Hemoterapia in 2015"

Hemoglobin D-Punjab: origin, distribution and laboratory diagnosis

Abstract: This review discusses hemoglobin D-Punjab, also known as hemoglobin D-Los Angeles, one of the most common hemoglobin variants worldwide. It is derived from a point mutation in the beta-globin gene (HBB: c.364G>C; rs33946267) prevalent in the Punjab region, Northwestern Indian. Hemoglobin D-Punjab can be inherited in heterozygosis with hemoglobin A causing no clinical or hematological alterations, or in homozygosis, the rarest form of inheritance, a condition that is commonly not related to clinical symptomatology. Moreover, this variant can exist in association with other hemoglobinopathies, such as thalassemias; the most noticeable clinical alterations occur when hemoglobin D-Punjab is associated to hemoglobin S. The clinical manifestations of this association can be similar to homozygosis for hemoglobin S. Although hemoglobin D-Punjab is a common variant globally with clinical importance especially in cases of double heterozygosis, hemoglobin S/D-Punjab is still understudied. In Brazil, for example, hemoglobin D-Punjab is the third most common hemoglobin variant. Thus, this paper summarizes information about the origin, geographic distribution, characterization and occurrence of hemoglobin D-Punjab haplotypes to try to improve our knowledge of this variant. Moreover, a list of the main techniques used in its identification is provided emphasizing the importance of complementary molecular analysis for accurate diagnosis.

Acute lymphoblastic leukemia in children and adolescents: prognostic factors and analysis of survival

Abstract: OBJECTIVE: To describe the clinical and laboratory features of children and adolescents with acute lymphoblastic leukemia treated at three referral centers in Ceara and evaluate prognostic factors for survival, including age, gender, presenting white blood cell count, immunophenotype, DNA index and early response to treatment.METHODS: Seventy-six under 19-year-old patients with newly diagnosed acute lymphoblastic leukemia treated with the Grupo Brasileiro de Tratamento de Leucemia da InfA¢ncia - acute lymphoblastic leukemia-93 and -99 protocols between September 2007 and December 2009 were analyzed. The diagnosis was based on cytological, immunophenotypic and cytogenetic criteria. Associations between variables, prognostic factors and response to treatment were analyzed using the chi-square test and Fisher's exact test. Overall and event-free survival were estimated by Kaplan-Meier analysis and compared using the log-rank test. A Cox proportional hazards model was used to identify independent prognostic factors.RESULTS: The average age at diagnosis was 6.3 ± 0.5 years and males were predominant (65%). The most frequently observed clinical features were hepatomegaly, splenomegaly and lymphadenopathy. Central nervous system involvement and mediastinal enlargement occurred in 6.6% and 11.8%, respectively. B-acute lymphoblastic leukemia was more common (89.5%) than T-acute lymphoblastic leukemia. A DNA index >1.16 was found in 19% of patients and was associated with favorable prognosis. On Day 8 of induction therapy, 95% of the patients had lymphoblast counts <1000/µL and white blood cell counts <5.0 A- 109/L. The remission induction rate was 95%, the induction mortality rate was 2.6% and overall survival was 72%.CONCLUSION: The prognostic factors identified are compatible with the literature. The 5-year overall and event-free survival rates were lower than those reported for developed countries. As shown by the multivariate analysis, age and baseline white blood cell count were independent prognostic factors.

Resistance of dialyzed patients to erythropoietin.

Abstract: 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.

Intravenous ferric carboxymaltose for the treatment of iron deficiency anemia.

Abstract: Nutritional iron deficiency anemia is the most common deficiency disorder, affecting more than two billion people worldwide. Oral iron supplementation is usually the first choice for the treatment of iron deficiency anemia, but in many conditions, oral iron is less than ideal mainly because of gastrointestinal adverse events and the long course needed to treat the disease and replenish body iron stores. Intravenous iron compounds consist of an iron oxyhydroxide core, which is surrounded by a carbohydrate shell made of polymers such as dextran, sucrose or gluconate. The first iron product for intravenous use was the high molecular weight iron dextran. However, dextran-containing intravenous iron preparations are associated with an elevated risk of anaphylactic reactions, which made physicians reluctant to use intravenous iron for the treatment of iron deficiency anemia over many years. Intravenous ferric carboxymaltose is a stable complex with the advantage of being non-dextran-containing and a very low immunogenic potential and therefore not predisposed to anaphylactic reactions. Its properties permit the administration of large doses (15mg/kg; maximum of 1000mg/infusion) in a single and rapid session (15-minute infusion) without the requirement of a test dose. The purpose of this review is to discuss some pertinent issues in relation to the history, pharmacology, administration, efficacy, and safety profile of ferric carboxymaltose in the treatment of patients with iron deficiency anemia.

Myeloproliferative neoplasms and the JAK/STAT signaling pathway: an overview

Abstract: Myeloproliferative neoplasms are caused by a clonal proliferation of a hematopoietic progenitor. First described in 1951 as 'Myeloproliferative Diseases' and reevaluated by the World Health Organization classification system in 2011, myeloproliferative neoplasms include polycythemia vera, essential thrombocythemia and primary myelofibrosis in a subgroup called breakpoint cluster region-Abelson fusion oncogene-negative neoplasms. According to World Health Organization regarding diagnosis criteria for myeloproliferative neoplasms, the presence of the JAK2 V617F mutation is considered the most important criterion in the diagnosis of breakpoint cluster region-Abelson fusion oncogene-negative neoplasms and is thus used as a clonal marker. The V617F mutation in the Janus kinase 2 (JAK2) gene produces an altered protein that constitutively activates the Janus kinase/signal transducers and activators of transcription pathway and other pathways downstream as a result of signal transducers and activators of transcription which are subsequently phosphorylated. This affects the expression of genes involved in the regulation of apoptosis and regulatory proteins and modifies the proliferation rate of hematopoietic stem cells.

Acute myeloid leukemia: survival analysis of patients at a university hospital of Paraná

Abstract: a b s t r a c t 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 Clinicas, Universidade Federal do Parana 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

Association between maternal and fetal factors and quality of cord blood as a source of stem cells

Abstract: a b s t r a c t 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 donations ranged from 71.6 to 275.2 mL, the total nucleated cell count ranged from 4.77 × 10 8 to

The compound state: Hb S/beta-thalassemia

Abstract: The polymerization of deoxygenated Hb S (sickling) is theprimary event in the molecular pathogenesis of SCD. How-ever, this event is highly dependent on the intracellular Hbcomposition; in other words, it is dependent on the concen-tration of Hb S, and type and concentration of the other typesof Hb. Therefore, the major primary genetic determinant ofthe severity of SCD is the genotype.

Socioeconomic and demographic characteristics of sickle cell disease patients from a low-income region of northeastern Brazil

Abstract: 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.

Experience with Evans syndrome in an academic referral center.

Abstract: OBJECTIVE: To document the experience of one referral service with patients diagnosed with Evans syndrome, the treatment and response and to briefly review current treatment strategies and results.METHODS: Patients enrolled in this study fulfilled criteria for Evans syndrome. Data were retrieved from the clinical files and electronic databases of the Department of Hematology, Hospital Universitario "Dr. Jose Eleuterio Gonzalez". Treatment modalities and response and the use of additional therapies were evaluated. The literature was reviewed in the context of the clinical course of the studied patients.RESULTS: Six patients were diagnosed with Evans syndrome in the study period. Patient 1 was treated with steroids, relapsed twice and was again treated with steroids. Patient 2 treated initially with steroids plus intravenous immunoglobulin was subsequently lost to follow-up. A good response was achieved in Patients 3 and 4, who were treated with steroids plus rituximab; patient 4 also received danazol as a second-line therapy. However both relapsed and subsequently underwent splenectomy at ten and nine months, respectively. One patient, number 5, treated with steroids, danazol and rituximab did not relapse within four years of follow-up and Patient 6, who received steroids plus danazol did not relapse within three years of follow-up.CONCLUSION: Evans syndrome is an uncommon hematologic condition rarely diagnosed and not widely studied. Clinicians must have it in mind when evaluating a patient with a positive direct antiglobulin test, anemia and thrombocytopenia, since prognosis depends on its early recognition and opportune therapy, but even this leads to variable results.

CD144, CD146 and VEGFR-2 properly identify circulating endothelial cell

Abstract: a b s t r a c t Studies evaluating circulating endothelial cells by flow cytometry are faced by a lack of consensus about the best combination of monoclonal antibodies to be used. The rarity of these cells in peripheral blood, which represent 0.01% of mononuclear cells, drastically increases this challenge. Objective: The aim of this study is to suggest some combinations of markers that would safely and properly identify these cells. Methods: Flow cytometry analysis of circulating endothelial cells was performed applying three different panels composed of different combinations of the CD144, CD146, CD31, CD133, CD45 and anti-Vascular endothelial growth factor receptor-2 antibodies. Results: In spite of the rarity of the events, they were detectable and presented similar interperson numbers of circulating endothelial cells. Conclusion: The combination of markers successfully identified the circulating endothelial cells in healthy individuals, with the use of three different panels confirming the obtained data as reliable.

Left ventricular hypertrophy in children, adolescents and young adults with sickle cell anemia.

Abstract: OBJECTIVE: The aims of this study were to estimate the frequency of left ventricular hypertrophy and to identify variables associated with this condition in under 25-year-old patients with sickle cell anemia.METHODS: A cross-sectional study was performed of children, adolescents and young adults with sickle cell anemia submitted to a transthoracic Doppler echocardiography. The mass of the left ventricle was determined by the formula of Devereux et al. with correction for height, and the percentile curves of gender and age were applied. Individuals with rheumatic and congenital heart disease were excluded. The patients were divided into two groups according to the presence or absence of left ventricular hypertrophy and compared according to clinical, echocardiographic and laboratory variables.RESULTS: A total of 37.6% of the patients had left ventricular hypertrophy in this sample. There was no difference between the groups of patients with and without hypertrophy according to pathological history or clinical characteristics, except possibly for the use of hydroxyurea, more often used in the group without left ventricular hypertrophy. Patients with left ventricular hypertrophy presented larger left atria and lower hemoglobin and hematocrit levels, reticulocyte index and a higher albumin:creatinine ratio in urine.CONCLUSION: Left ventricular hypertrophy was observed in more than one-third of the young patients with sickle cell anemia with this finding being inversely correlated to the hemoglobin and hematocrit levels, and reticulocyte index and directly associated to a higher albumin/creatinine ratio. It is possible that hydroxyurea had had a protective effect on the development of left ventricular hypertrophy.

Trends in mortality and hospital admissions of sickle cell disease patients before and after the newborn screening program in Maranhão, Brazil

Abstract: 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 Maranhao, Brazil. Methods: A descriptive study was performed of all inpatients and deaths of patients with a diagnosis of sickle cell disease in Maranhao 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 Maranhao 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 Maranhao.

Transfusion of older red blood cell units, cytokine burst and alloimmunization: a case–control study

Abstract: BACKGROUND: Experimental data have shown that the transfusion of older red blood cell units causes alloimmunization, but the clinical applicability of this statement has never been properly assessed in non-sickle cell patients. It has been hypothesized that older units have higher numbers of cytokines, increasing the risk of alloimmunization related to antigen-presenting events. The goal of this study was to evaluate the association between the transfusion of older red blood cell units subjected to bedside leukodepletion and alloimmunization.METHODS: All patients submitted to transfusions of bedside leukodepletion red blood cell units proven to have become alloimmunized in one oncologic service between 2009 and 2013 were enrolled in this study. A control group was formed by matching patients without alloimmunization in terms of number of transfusions and medical specialty. The median age of transfused units, the percentage of transfused red blood cell units >14 days of storage in relation to fresher red cell units (≤14 days of storage) and the mean age of transfused units older than 14 days were compared between the groups.RESULTS: Alloimmunized and control groups were homogeneous regarding the most relevant clinical variables (age, gender, type of oncological disease) and inflammatory background (C-reactive protein and Karnofsky scale). The median age of transfused red blood cell units, the ratio of older units transfused compared to fresher units and the mean age of transfused units older than 14 days did not differ between alloimmunized and control patients (17 vs. 17; 68/32 vs. 63.2/36.8 and 21.8 ± 7.0 vs. 21.04 ± 7.9; respectively).CONCLUSION: The transfusion of older red blood cell units subjected to bedside leukodepletion is not a key risk factor for alloimmunization. Strategies of providing fresh red cell units aiming to avoid alloimmunization are thus not justified.

Teaching transfusion medicine: current situation and proposals for proper medical training

Abstract: 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 services 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.

Pattern of hemolysis parameters and association with fetal hemoglobin in sickle cell anemia patients in steady state

Abstract: 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, Ceara, 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 <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 <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.

Pilot randomized controlled trial to evaluate the effect of aquatic and land physical therapy on musculoskeletal dysfunction of sickle cell disease patients

Abstract: Objective: To compare the effect of aquatic and land-based physiotherapy in reducing musculoskeletal hip and lower back pain and increasing overall physical capabilities of sickle cell disease patients. Methods: Informed written consent was obtained from all volunteers who were submitted to evaluations using different functional scales: Lequesne's Algofunctional Questionnaire and Oswestry Disability Index, trunk and hip range of motion, goniometry, trunk and hip muscle strength assessment using load cell, and surface electromyography of the iliocostalis, long dorsal (longissimus), gluteus maximus, gluteus medius and tensor fasciae latae muscles. Ten patients were randomized into two groups: aquatic physiotherapy with a mean age of 42 years (range: 25-67) and conventional physiotherapy with a mean age of 49 years (range: 43-59). Both groups were submitted to a twelve-week program of two sessions weekly. Results: After the intervention, significant improvements were observed regarding the Lequesne index (p-value = 0.0217), Oswestry Disability Index (p-value = 0.0112), range of motion of trunk extension (p-value = 0.0320), trunk flexion muscle strength (p-value = 0.0459), hip extension and abduction muscle strength (p-value = 0.0062 and p-value = 0.0257, respec- tively). Range of motion of trunk and hip flexion, extension, adduction and abduction, trunk extensor muscle strength and all surface electromyography variables showed no significant statistical difference. Conclusion: Physical therapy is efficient to treat musculoskeletal dysfunctions in sickle cell disease patients, irrespective of the technique; however, aquatic therapy showed a trend toward improvement in muscle strength. Further studies with a larger patient sample and longer periods of therapy are necessary to confirm these results.

Prevalence of the American College of Rheumatology hematological classification criteria and associations with serological and clinical variables in 460 systemic lupus erythematosus patients.

Abstract: Objective: To study systemic lupus erythematosus in a Brazilian population using the American College of Rheumatology hematological classification criteria and report associations of the disease with serological and clinical profiles. Methods: This is a retrospective study of 460 systemic lupus erythematosus patients followed in a single rheumatologic center during the last 10 years. Hematological manifestations considered for this study were hemolysis, leukopenia, lymphocytopenia and thrombocytopenia. Results: The cumulative prevalences of leukopenia, thrombocytopenia, lymphocytopenia and hemolytic anemia were 29.8%, 21.08%, 17.7% and 8.4%, respectively. A higher percentage of patients with hemolysis had anticardiolipin IgM (p-value = 0.002). Those with leukopenia had more lymphopenia (p-value = 0.02), psychosis (p-value = 0.01), thrombocy- topenia (p-value <0.0001) and anti-double stranded DNA antibodies (p-value = 0.03). Patients with lymphopenia had more leukopenia (OR = 1.8; 95% CI = 1.01-3.29) and lupus anticoagulant antibodies (OR = 2.2; 95% CI = 1.16-4.39) and those with thrombocytopenia had more leukopenia (OR = 3.1; 95% CI = 1.82-5.44) and antiphospholipid syndrome (OR = 3.1; 95% CI = 1.28-7.87). Conclusion: The most common hematological finding was leukopenia and the least common was hemolysis. Associations of low platelet count and hemolysis were found with antiphospholipid syndrome and anticardiolipin IgM positivity, respectively. Leukopenia and lymphocytopenia are correlated and leukopenia is more common in systemic lupus erythe- matosus patients with psychosis, thrombocytopenia and anti-double stranded DNA.

Prevalence of anemia in a sample of elderly southeastern Brazilians

Abstract: Objective: To demonstrate the proportion of anemia and its association with demographic and clinical characteristics in a representative sample of elderly people from Sao Jose dos Campos, Sao 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 75-80, >85-90 and >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 Sao Jose 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.

Results of high-risk neutropenia therapy of hematology-oncology patients in a university hospital in Uruguay

Abstract: 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.

Torque teno virus: a ubiquitous virus

Abstract: The TTV genome can be divided into an untranslatedregion (UTR) of 1.2kb and a potential coding region of 2.6kb.The UTR is relatively conserved, suggesting that it playsan important regulatory role in viral replication. The cod-ing region contains two large open reading frames: ORF1 andORF2. Several other open reading frames have been described,and the peptides that they encode differ in length for differentisolates.

Evaluation of the effectiveness of packed red blood cell irradiation by a linear accelerator

Abstract: 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 >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.

Hemoglobin A2 values in sickle cell disease patients quantified by high performance liquid chromatography and the influence of alpha thalassemia.

Abstract: a b s t r a c t Background: In sickle cell disease, the quantification of Hb A2 is important for the differential

Identifying the similarities and differences between single nucleotide polymorphism array (SNPa) analysis and karyotyping in acute myeloid leukemia and myelodysplastic syndromes

Abstract: 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.

Primary nodal peripheral T-cell lymphomas: diagnosis and therapeutic considerations

Abstract: a b s t r a c t Nodal peripheral T-cell lymphomas are a rare group of neoplasms derived from post-thymic and activated T lymphocytes. A review of scientific articles listed in PubMed, Lilacs, and the Cochrane Library databases was performed using the term “peripheral T-cell lymphomas”. According to the World Health Organization classification of hematopoietic tissue tumors, this group of neoplasms consists of peripheral T-cell lymphoma not otherwise specified (PTCL-NOS), angioimmunoblastic T-cell lymphoma (AITL), anaplastic large cell lymphoma-anaplastic lymphoma kinase positive (ALCL-ALK + ), and a provisional entity called anaplastic large cell lymphoma-anaplastic lymphoma kinase negative (ALCL-ALK

Prevalence of Torque teno virus in healthy donors of Paraná State, southern Brazil

Abstract: OBJECTIVE: To determine the prevalence of the Torque teno virus in healthy donors in the northern and northwestern regions of the state of Parana, southern Brazil.METHODS: The Torque teno virus was detected by a nested polymerase chain reaction using a set of oligoprimers for the N22 region.RESULTS: The prevalence of the virus was 69% in 551 healthy blood donors in southern Brazil. There was no statistically significant difference between the presence of the virus and the variables gender, ethnicity and marital status. There was significant difference in the prevalence of the virus regarding the age of the donors (p-value = 0.024) with a higher incidence (74.7%) in 18- to 24-year-old donors.CONCLUSION: A high prevalence of Torque teno virus was observed in the population studied. Further studies are needed to elucidate the routes of contamination and the clinical implications of the virus in the healthy population.

Remission of refractory Crohn's disease after autologous hematopoietic stem cell transplantation

Abstract: The etiology of CD is unknown, but several proven riskfactors exist such as family history, smoking, oral contra-ceptives, diet and ethnicity The combination of risk factors,and aberrant autoimmune response in the intestinal mucosawith endothelial dysfunction lead to digestive tract malfunc-tioning Disease manifestations are heterogeneous but mostpatients present with abdominal pain, diarrhea sometimeswith blood, weight loss, fever and perianal lesions Up to 30%of

Proposal for the standardization of flow cytometry protocols to detect minimal residual disease in acute lymphoblastic leukemia

Abstract: Minimal residual disease is the most powerful predictor of outcome in acute leukemia and is useful in therapeutic stratification for acute lymphoblastic leukemia protocols. Nowadays, the most reliable methods for studying minimal residual disease in acute lymphoblastic leukemia are multiparametric flow cytometry and polymerase chain reaction. Both provide similar results at a minimal residual disease level of 0.01% of normal cells, that is, detection of one leukemic cell in up to 10,000 normal nucleated cells. Currently, therapeutic protocols establish the minimal residual disease threshold value at the most informative time points according to the appropriate methodology employed. The expertise of the laboratory in a cancer center or a cooperative group could be the most important factor in determining which method should be used. In Brazil, multiparametric flow cytometry laboratories are available in most leukemia treatment centers, but multiparametric flow cytometry processes must be standardized for minimal residual disease investigations in order to offer reliable and reproducible results that ensure quality in the clinical application of the method. The Minimal Residual Disease Working Group of the Brazilian Society of Bone Marrow Transplantation (SBTMO) was created with that aim. This paper presents recommendations for the detection of minimal residual disease in acute lymphoblastic leukemia based on the literature and expertise of the laboratories who participated in this consensus, including pre-analytical and analytical methods. This paper also recommends that both multiparametric flow cytometry and polymerase chain reaction are complementary methods, and so more laboratories with expertise in immunoglobulin/T cell receptor (Ig/TCR) gene assays are necessary in Brazil.

Health-related quality of life among blood donors with hepatitis B and hepatitis C: longitudinal study before and after diagnosis

Abstract: Introduction: There is evidence that patients suffering from chronic hepatic diseases, including chronic hepatitis B and chronic hepatitis C, have a reduced health-related quality of life. The aim of this study was to evaluate the impact of the notification of test results for hepatitis B and hepatitis C on the quality of life of blood donors. Methods: Over a 29-month period, this study assessed the quality of life of 105 blood donors with positive serological screening tests for hepatitis B and hepatitis C and donors who presented false-positive test results. The Medical Outcome Study 36-Item Short Form Health Survey Questionnaire was applied at three time points: (1) when an additional blood sample was collected for confirmatory tests; (2) when donors were notified about their serological status; and (3) when donors, positive for hepatitis B and hepatitis C, started clinical follow- up. Quality of life scores for the confirmed hepatitis B and hepatitis C groups were compared to the false-positive control group. Results: The domains bodily pain, general health perception, social function, and mental health and the physical component improved significantly in donors with hepatitis C from Time Point 1 to Time Point 3. Health-related quality of life scores of donors diagnosed with hepatitis B and hepatitis C were significantly lower in six and four of the eight domains, respectively, compared to the false-positive control group. Conclusion: A decreased quality of life was detected before and after diagnosis in blood donors with hepatitis B and hepatitis C. Contrary to hepatitis B positive donors, the pos- sibility of medical care may have improved the quality of life among hepatitis C positive donors.

Haptoglobin gene polymorphisms and interleukin-6 and -8 levels in patients with sickle cell anemia

Abstract: BACKGROUND: Haptoglobin genotypes, and interleukin-6 and -8 participate in the pathophysiology of sickle cell anemia. The expression of cytokines is regulated by genetic mechanisms however the effect of haptoglobin polymorphisms on these cytokines is not fully understood. This study aimed to compare the frequency of haptoglobin genotypes and the interleukin-6 and -8 concentrations in sickle cell anemia patients and controls to investigate the association between haptoglobin genotypes and cytokine levels.METHODS: Sixty sickle cell anemia patients and 74 healthy individuals were analyzed. Haptoglobin genotypes were determined by multiplex polymerase chain reaction, and the interleukin-6 and -8 levels by enzyme linked immunosorbent assay. The association between haptoglobin genotypes and cytokines was investigated by statistical tests.RESULTS:Hp2-1 was the most common genotype in both the cases and controls while Hp1-1 was less frequent among sickle cell anemia patients. Interleukin-6 and -8 levels were higher in patients than controls (p-value 0.05). A similar trend was observed among the controls.CONCLUSION: Although, levels of interleukin-6 and -8 were higher in the sickle cell anemia patients, they appeared not to be related to the haptoglobin genotypes. Further investigations are necessary to identify factors responsible for increased secretion of the interleukin-6 and -8 pro-inflammatory cytokines in patients with sickle cell anemia.