Resumo em Inglês:

BACKGROUND: With subclinical infection, serum iron concentrations are reduced, altering the synthesis of hemoglobin, the main indicator of anemia. OBJECTIVE: To evaluate the association between subclinical infection and anemia in children of Paraíba State. METHODS: This is a cross-sectional study involving 1116 children aged 6 to 59 months from nine municipalities of Paraíba State. Demographic and socioeconomic data were collected by means of a specific questionnaire. The C-reactive protein and hemoglobin levels were determined by the latex agglutination technique and automated counter, respectively. C-reactive protein values > 6 mg/L were used as indicative of subclinical infection, while the presence of anemia was determined by hemoglobin values < 11.0 g/dL. The data were analyzed using the Epi Info computer program, with significance being set at 5%. RESULTS: Data from this research showed that 80.1% of the children belonged to families that were below the bread line, with per capita income < ½ of the minimum wage at that time (R$ 350.00 approximately US$ 175.00). The prevalences of subclinical infection and anemia were 11.3% and 36.3%, respectively. Subclinical infection was significantly associated with anemia (p-value < 0.05). There were lower levels of hemoglobin in children with C-reactive protein > 6 mg/L, with a mean hemoglobin level in children with subclinical infection of 10.93 g/dL (standard deviation - SD = 1.21 g/dL) and without infection of 11.26 g/dL (SD = 1.18 g/dL) (p-value < 0.05). CONCLUSION: Anemia is associated with subclinical infection in this population, indicating that this is an important variable to be considered in studies of the prevalence of anemia in children.

Resumo em Inglês:

INTRODUCTION: Anemia is a nutritional problem of global importance. It is estimated that at least one-third of the population has been at one time anemic. OBJECTIVE: To evaluate the prevalence of anemia and associated factors in patients of a children's hospital in Recife. METHODS: A cross-sectional study was developed involving 595 male and female children aged from 6 to 59 months old, who were hospitalized in 2007. RESULTS: Children with a hemoglobin concentration less than 11 g/dL were considered anemic. The relationship between studied variables and anemia was evaluated by Poisson regression analysis. There was a 56.6% prevalence of anemia (95% CI: 46.6-54.6). Anemia was significantly correlated with low weight (Prevalence ratio - PR = 1.39; 95% CI: 1.18-1.64), young age (PR = 2.01; 95% CI: 1.57-2.56) and a diagnosis of acute lower respiratory disease (PR = 1.57; 95% CI: 1.27-1.96). CONCLUSION: The high prevalence of anemia suggests that it may contribute as a causal factor for hospitalization, especially because the period of hospitalization was short and the patient was likely to be anemic at the time of admission. This study stresses the importance of evaluating the overall nutritional status of patients, including their ingestion of microelements. This is especially important in children, because of their greater susceptibility to anemia. Measures directed at the prevention and control of anemia, including increased coverage of supplementation and fortification programs are strongly recommended.

Resumo em Inglês:

BACKGROUND: The development of therapies for sickle cell disease has received special attention, particularly those that reduce the polymerization of hemoglobin S. Hydroxyurea is a commonly used medication because it has the ability to raise levels of fetal hemoglobin, decrease the frequency of vaso-occlusive episodes and thus improve the clinical course of sickle cell disease patients. OBJECTIVE: To study hematological data and the clinical profile of sickle cell disease patients taking hydroxyurea in a regional blood center. METHODS: From the charts of 20 patients with sickle cell anemia, the clinical outcomes and a number of hematological variables were analyzed before and during treatment with hydroxyurea. RESULTS: The patients' ages ranged from 6 to 41 years old, most were dark skinned and there was a predominance of women. The main symptom that defined whether patients were prescribed hydroxyurea was painful crises followed by hospitalizations. During treatment with hydroxyurea there were significant increases in hemoglobin, fetal hemoglobin, mean corpuscular volume and mean corpuscular hemoglobin. The reticulocyte and white blood cell counts dropped significantly with treatment. A positive correlation was found between fetal hemoglobin and mean corpuscular volume before and during treatment. Additionally, a correlation was found between the white blood cell and reticulocyte counts before treatment with hydroxyurea. CONCLUSION: Most patients showed improvements with treatment as demonstrated by increases in hemoglobin, fetal hemoglobin and mean corpuscular volume, as well as by reductions in the reticulocyte and white blood cell counts. Clinically, more than 50% of patients had a significant reduction of events.

Resumo em Inglês:

BACKGROUND: The study of platelet aggregation is essential to assess in vitro platelet function by different platelet activation pathways. OBJECTIVE: To assess aggregation and biochemical parameters of random platelet concentrates produced at the Fundação HEMOAM using the quality control tests defined by law. METHODS: Whole blood samples from 80 donors and the respective platelet concentrate units were tested. Platelet concentrates were tested (platelet count, aggregation and pH) on days 1, 3 and 5 of storage. Additionally a leukocyte count was done only on day 1 and microbiological tests on day 5 of storage. Collagen and adenosine diphosphate were used as inducing agonists for platelet aggregation testing. RESULTS: Donor whole blood had normal aggregation (aggregation with adenosine diphosphate = 67% and with collagen = 78%). The median aggregation in platelet concentrates with adenosine diphosphate was low throughout storage (18% on day 1, 7% on day 3 and 6% on day 5) and the median aggregation with collagen was normal only on day 1 and low thereafter (54.4% on day 1, 20.5% on day 3 and 9% on day 5). CONCLUSION: Although the results were within the norms required by law, platelet concentrates had low aggregation rates. We suggest the inclusion of a functional assessment test for the quality control of platelet concentrates for a more effective response to platelet replacement therapy.

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Hemoglobinopathies, in particular sickle cell disease, is the most prevalent group of genetically transmitted diseases in the Brazilian population and should thus be treated as a public health problem. Many of these patients frequently present with complications and require emergency care at the blood bank Coordinator in Rio de Janeiro. This study was developed with the aim of characterizing the emergency assistance required by sickle cell disease patients registered in the blood bank from January 2007 to December 2008. A retrospective study of medical records was made of 78, mostly children, patients from the date of their registration until December 2009. Most attendances (63.7%) were not considered emergency care. The use of specialized services for cases that do not require this level of complexity may saturate the capacity of these centers. However, delay of intervention for complications due to the transportation of patients to specialist centers may lead to deterioration in the clinical condition.

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BACKGROUND: Based on the necessity of detailed information that supports effective strategies to improve cancer outcomes in the different regions of Brazil, the aims of this study were to report demographic aspects and to calculate the prevalence and incidence rates of oncohematological diseases in the region of Vale do Paraíba. METHODS: This is a multicentric prospective study carried out from October 2009 to March 2010. A total of 500 over 19-year-old patients were enrolled. Data such as type of healthcare insurance, gender, age, ethnic classification, place of residence, schooling, income, body mass index, new cases and the period between the first symptoms and a definite diagnosis were collected. The prevalence and incidence rates were calculated according to an estimated number of 1,319,800 inhabitants. RESULTS: The prevalence and incidence rates per 100,000 inhabitants in the period of six months were, respectively: acute myeloid leukemia 1.5 and 0.7; acute lymphoblastic leukemia 0.5 and 0.1; chronic lymphocytic leukemia 2.4 and 0.4; chronic myeloid leukemia 6.2 and 0.8; Hodgkin's lymphoma 2.9 and 0.9; non-Hodgkin lymphoma 9.8 and 4.3; multiple myeloma 5.7 and 0.7; myelodysplastic syndromes 2.1 and 0.2 and myeloproliferative syndromes 5.1 and 0.3. CONCLUSION: Giving the paucity of data in this field of investigation, our data may be useful for comparisons with those of other regions of Brazil and will assist in the implementation of treatment programs of oncohematological diseases in this region.

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Hematopoietic stem cell transplantation is the treatment of choice for many hematologic diseases, such as multiple myeloma, bone marrow aplasia and leukemia. Human leukocyte antigen (HLA) compatibility is an important tool to prevent post-transplant complications such as graft rejection and graft-versus-host disease, but the high rates of relapse limit the survival of transplant patients. Natural Killer cells, a type of lymphocyte that is a key element in the defense against tumor cells, cells infected with viruses and intracellular microbes, have different receptors on their surfaces that regulate their cytotoxicity. Killer immunoglobulin-like receptors are the most important, interacting consistently with human leukocyte antigen class I molecules present in other cells and thus controlling the activation of natural killer cells. Several studies have shown that certain combinations of killer immunoglobulin-like receptors and human leukocyte antigens (in both donors and recipients) can affect the chances of survival of transplant patients, particularly in relation to the graft-versusleukemia effect, which may be associated to decreased relapse rates in certain groups. This review aims to shed light on the mechanisms and effects of killer immunoglobulin-like receptors - human leukocyte antigen associations and their implications following hematopoietic stem cell transplantation, and to critically analyze the results obtained by the studies presented herein.

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Dasatinib is a highly effective second generation tyrosine kinase inhibitor approved for the treatment of imatinib-resistant or intolerant chronic myeloid leukemia and Philadelphia-positive acute lymphoblastic leukemia. This article reviews the results of phase I, II and III studies and looks at the efficacy and safety of dasatinib. This review also provides practical recommendations for the management of side effects.

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Lymphomas are a heterogeneous group of malignancies that have a distinct biological behavior according to the subtype and degree of differentiation. Adequate staging, which has a direct impact on prognosis, is essential to properly plan therapy. Structural cross-sectional imaging, such as computed tomography, has been the standard imaging tool to stage and monitor patients with lymphoma. However, molecular imaging such as positron emission tomography has shown complementary diagnostic and prognostic values. This review discusses the current value of positron emission tomography imaging using 2-[fluorine-18]fluoro-2-deoxy-d-glucose in staging, restaging, monitoring and detecting relapse in Hodgkin's and non-Hodgkin lymphoma.

Resumo em Inglês:

A case of a follicular lymphoma transformed into a CD20+ is described which progressed with the loss of CD20 expression after 8 cycles of R-CHOP. This phenomenon is not a rare event and has shown poor prognosis. Our purposes are to describe this event and suggest biopsy in relapsed or progressive disease.

Resumo em Inglês:

The 22q11.2 deletion syndrome (22q11DS) is a common genetic disease characterized by broad phenotypic variability. Despite the small number of studies describing hematological alterations in individuals with 22q11DS, it appears that these abnormalities are more frequent than previously imagined. Thus, the objective of our study was to report on a patient with 22q11DS presenting thrombocytopenia and large platelets and to review the literature. The patient, a 13-year-old boy, was originally evaluated due to craniofacial dysmorphia and speech delay. He also had a history of behavioral changes, neuropsychomotor delay and recurrent otitis/sinusitis. The identification of a 22q11.2 microdeletion by fluorescent in situ hybridization diagnosed the syndrome. Despite his hematological alterations, he only had a history of epistaxis and bruising of the upper and lower limbs. Assessments of the prothrombin time, thrombin time, partial thromboplastin time, bleeding time, fibrinogen levels and platelet aggregation (including the ristocetin induced platelet aggregation test) were all normal. Hematological alterations observed in 22q11DS are directly related to the genetic disorder itself (especially in respect to deletion of the GPIb gene) and secondary to some clinical findings, such as immunodeficiency. Macrothrombocytopenia is increasingly being considered a feature of the broad spectrum of 22q11DS and may potentially be a clinical marker for the syndrome.

Resumo em Inglês:

A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death.

Resumo em Inglês:

Government health authorities approved, in December 2002, the ANVISA (National Sanitary Vigilance Agency) resolution number 344, making the addition of iron and folic acid to all wheat and maize flours industrialized in Brazil obligatory. After a brief review of iron deficiency, iron overload and folic acid deficiency several questions and remarks need to be made about this universal food fortification program. Iron salts and folic acid are drugs widely used in medicine and they may present undesirable side effects. There are potential risks with offering iron to the normal population for a long period of time and to patients with iron overload. Other important remarks are: there is no medical follow up of this treatment in the Brazilian population; patients can decide the quantity of foods (and of these nutrients) that they want to ingest; fortified foods may correct iron deficiency anemia but not necessarily the causes, which include gastrointestinal neoplasms; and folic acid in the diet may interfere with several treatment protocols that use folic acid antagonists, such as methotrexate. Finally, with the exception of some social programs, the costs of treatment using fortified foods are passed on to the population. Considering that Brazil has 330,000 active medical doctors it is suggested that our Health Ministry should invite them to take care of these important medical conditions.