Resumo em Inglês:

BACKGROUND: Hereditary spherocytosis (HS) is an inherited hemolytic anemia that is caused by deficiency or dysfunction of erythrocyte cytoskeletal proteins. AIM: The aim of this study was to describe the clinical course of hereditary spherocytosis in patients treated in the Pediatric Hematology Unit, Hospital das Clínicas, Universidade Federal de Minas Gerais. METHODS: Sixty-three under 16-year-old patients with hereditary spherocytosis were retrospectively evaluated between January 1988 and December 2007. Hereditary spherocytosis was diagnosed based on clinical history, physical examination and on a positive osmotic fragility curve. Patients underwent screening for cholelithiasis by ultrasonography. They were classified into three groups: mild, moderate and severe. The events of interest were need for blood transfusion, cholelithiasis, splenic sequestration, aplastic crisis, and splenectomy. Differences between subgroups were evaluated by the two-sided log-rank test. RESULTS: The mean age at diagnosis was 5.2 years and most patients were classified as moderate (54%). Patients with the severe form of the disease were younger (p-value = 0.001) and needed more blood transfusions (p-value = 0.004). Seventeen patients (27%) developed cholelithiasis, 14 (22.2%) splenic sequestration and three (4.8%) aplastic crises. Twenty-two patients (34.9%) were splenectomized with the main indication being splenic sequestration in nine patients (41%). CONCLUSIONS: The clinical course of patients with hereditary spherocytosis in this study was relatively benign however cholelithiasis was a common complication.

Resumo em Inglês:

OBJECTIVE: The aim of this work was to establish reference values for methemoglobin levels in 6 to 10-year-old children. METHODS: Methemoglobin concentrations were studied in clinically healthy children. The method for methemoglobin measurement used, neither uses highly toxic chemical compounds nor expensive enzymatic methods, thus it is feasible in the laboratory routine. RESULTS: The results showed higher reference values for clinically healthy children (from 3.61 to 6.44%) than for adults (from 1.9 to 3.8%). CONCLUSIONS: The higher concentrations of methemoglobin in children may be explained by smaller amounts of soluble cofactor cytochrome b5 and reduced activity of the cytochrome b5 reductase enzyme in red blood cells which make children particularly susceptible to the development of methemoglobinemia. Methemoglobin concentrations in children are higher than in normal adult subjects thus, adult reference values cannot be used to interpret infant methemoglobinemia.

Resumo em Inglês:

OBJECTIVE: This study aimed at analyzing the rate of self-exclusion at the Regional Blood Bank in Montes Claros. METHODS: Data of self-excluding donors from August 2008 to August 2010 were analyzed. The following variables were considered: age, marital status, gender, ethnical background, blood group, Rh factor, number of donations, type of donation and serologic results. RESULTS: During the analyzed period, 34,778 individuals donated blood, 215 (0.62%) of which were self-excluded; 12% of donors did not answer, 6.3% ballots were spoilt and 13.6% of the responses were considered non-compliant. The profile of the donors was: male (81.9%), single (50.7%), aged between 19 and 29 years old (52.1%), Mulatto (48.3%), blood group O (32.1%) and positive Rh (32.1%). Most individuals were donating for the 2nd to 5th time (43.7%) and had negative serology (94.4%). CONCLUSIONS: It was not evident that self-excluding donors had higher rates of seropositivity.

Resumo em Inglês:

OBJECTIVE: This study aimed at identifying associations between the participation of transfusion services in immunohematology external quality control programs and their accuracy in immunohematology testing and adaptation to technical and legal operational procedures. METHODS: From 2007 to 2009, a cross-sectional study was conducted in 219 transfusion services of the State of Minas Gerais who participated in this investigation by responding to a questionnaire and conducting a proficiency test comprising: ABO and RhD phenotyping, irregular RBC antibody screening and cross-matching. Frequencies and bivariate analysis followed by binary logistic regression were used for statistical analysis. RESULTS: Transfusion services who participated in external quality control programs (32.4%) and those that did not (67.6%) obtained worrying error percentages in proficiency tests which may significantly increase blood transfusion risks. Shortfalls related to the establishment of protocols, standards and internal quality control were also significant. On comparing the two groups, transfusion services that participated in these programs had a 2.35 times higher chance of correct results in the proficiency panel testing, a 3.16 higher chance of having transfusional records and a 2.81 higher chance of performing preventive maintenance of equipment. CONCLUSIONS: The study showed that independent factors associated to participation in external quality control programs suggest that more investment in internal quality control procedures is necessary and that more attention should be paid to current legislation.

Resumo em Inglês:

BACKGROUND: Knowledge of allele and haplotype frequencies of the human leukocyte antigen (HLA) system is important in the search for unrelated bone marrow donors. The Brazilian population is very heterogeneous and the HLA system is highly informative of populations because of the high level of polymorphisms. AIM: The aim of this study was to characterize the immunogenetic profile of ethnic groups (Caucasians, Afro-Brazilians and Asians) in the north of Parana State. METHODS: A study was carried out of 3978 voluntary bone marrow donors registered in the Brazilian National Bone Marrow Donor Registry and typed for the HLA-A, B and DRB1 (low resolution) loci. The alleles were characterized by the polymerase chain reaction sequence-specific oligonucleotides method using the LabType SSO kit (One Lambda, CA, USA). The ARLEQUIN v.3.11 computer program was used to calculate allele and haplotype frequencies. Results: The most common alleles found in Caucasians were HLA-A*02, 24, 01; HLA-B*35, 44, 51; DRB1*11, 13, 07; for Afro-Brazilians they were HLA-A*02, 03, 30; HLA-B*35, 15, 44; DRB1*13, 11, 03; and for Asians they were: HLA-A*24, 02, 26; HLA-B*40, 51, 52; DRB1*04, 15, 09. The most common haplotype combinations were: HLA-A*01, B*08, DRB1*03 and HLA-A*29, B*44, DRB1*07 for Caucasians; HLA-A*29, B*44, DRB1*07 and HLA-A*01, B*08 and DRB1*03 for Afro-Brazilians; and HLA-A*24, B*52, DRB1*15 and HLA-A*24, B*40 and DRB1*09 for Asians. CONCLUSIONS: There is a need to target and expand bone marrow donor campaigns in the north of Parana State. The data of this study may be used as a reference by the Instituto Nacional de Cancer/Brazilian National Bone Marrow Donor Registry to evaluate the immunogenetic profile of populations in specific regions and in the selection of bone marrow donors.

Resumo em Inglês:

OBJECTIVE: A previous study by our research group evaluated the levels of DNA damage using the comet assay in hemodialysis patients with type 2 diabetes mellitus. The same blood samples were also evaluated using the cytochalasin B micronucleus assay. A comparison of the results of the two assays is presented here. METHODS: Whole blood samples were collected from 22 type 2 diabetes mellitus patients on hemodialysis and from 22 control subjects. Samples were collected from patients early in the morning on Mondays, before the first weekly hemodialysis session. The cytokinesis-block micronucleus assay (CBMN) was used to evaluate genomic instability. RESULTS: The frequencies of micronuclei and nuclear buds were higher in patients than in controls (p-value = 0.001 and p-value < 0.001, respectively). There was a correlation between the frequency of micronuclei and DNA damage with the results of the comet assay (p-value < 0.001). The difference in the frequency of micronuclei and nuclear buds between patients and controls was more pronounced in the group with higher median comet values than in the group with lower comet values. CONCLUSIONS: Our results suggest that the increased rates of DNA damage as measured by the comet assay and influenced by the weekly routine therapy of these patients has a mutagenic effect, thereby increasing the risk of cancer in this group.

Resumo em Inglês:

AIM: The objective of this study was to correlate laboratory tests during the evolution of dengue fever, comparing frequencies between the different clinical forms in order to use test results to predict the severity of the disease. METHODS: This is an observational, descriptive and retrospective study of 154 patients with clinical and serological diagnoses of dengue fever who, in the period from January to May 2008, were admitted in a tertiary state hospital in the city of Fortaleza that is a referral center for infectious diseases. The patients were allocated to two groups according to age: under 15 years old (n = 66) and 15 years or older (n = 88). The tests analyzed were blood count, platelet count, and serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) concentrations. RESULTS: Thrombocytopenia and elevated transaminases were observed in patients with classic dengue fever. The main laboratory abnormalities found in dengue hemorrhagic fever were thrombocytopenia, hemoconcentration and elevated transaminases, similar to severe dengue with the exception of hemoconcentration. Most laboratory abnormalities started on the 3rd day but were more evident on the 5th day with restoration of values by the 11th day; this was more prominent in under 15-year-olds and with the more severe clinical forms. CONCLUSIONS: These results are relevant in assessing the disease because they can be used as markers for more severe forms and can help by enabling the adaptation of the therapeutic conduct to the needs of individual patients.

Resumo em Inglês:

Peripheral T-cell lymphomas are a group of rare neoplasms originating from clonal proliferation of mature post-thymic lymphocytes with different entities having specific biological characteristics and clinical features. As natural killer cells are closely related to T-cells, natural killer-cell lymphomas are also part of the group. The current World Health Organization classification recognizes four categories of T/natural killer-cell lymphomas with respect to their presentation: disseminated (leukemic), nodal, extranodal and cutaneous. Geographic variations in the distribution of these diseases are well documented: nodal subtypes are more frequent in Europe and North America, while extranodal forms, including natural killer-cell lymphomas, occur almost exclusively in Asia and South America. On the whole, T-cell lymphomas are more common in Asia than in western countries, usually affect adults, with a higher tendency in men, and, excluding a few subtypes, usually have an aggressive course and poor prognosis. Apart from anaplastic lymphoma kinase-positive anaplastic large cell lymphoma, that have a good outcome, other nodal and extranodal forms have a 5-year overall survival of about 30%. According to the principal prognostic indexes, the majority of patients are allocated to the unfavorable subset. In the past, the rarity of these diseases prevented progress in the understanding of their biology and improvements in the efficaciousness of therapy. Recently, international projects devoted to these diseases created networks promoting investigations on T-cell lymphomas. These projects are the basis of forthcoming cooperative, large scale trials to detail biologic characteristics of each sub-entity and to possibly individuate targets for new therapies.

Resumo em Inglês:

Currently, Hodgkin's lymphoma is one of the most curable types of cancer. Patients are often young and so the long-term morbidities of treatment have become of increasing concern. Among these, infertility is one of the most challenging consequences for patients in reproductive age. Premature ovarian failure in premenopausal women is a serious long-term sequel of the toxicity of chemotherapy. The main consequence of this syndrome is infertility, but women also present other symptoms related to estrogen deprivation. Different rates of impaired gonadal function are reported, depending on the patient's age, stage of disease, dose and intensity of chemotherapy and the use of radiation therapy. The most established strategy in female infertility is cryopreservation of embryos after in vitro fertilization. Additionally, the use of oral contraceptives or gonadotropinreleasing hormone analogs (GnRH-a) during treatment is under study. This review will provide a general overview of the main studies conducted to evaluate the infertility rate among female Hodgkin's lymphoma survivors and risk factors associated to treatment, different end-point definitions for evaluating fertility and also a brief description of the available strategies for fertility preservation.

Resumo em Inglês:

Follicular lymphoma is the second most frequent non-Hodgkin lymphoma accounting for about 10-20% of all lymphomas in western countries. The median age at diagnosis is 60 years old. The clinical presentation is usually characterized by asymptomatic peripheral adenopathy in cervical, axillary, inguinal and femoral regions. Treatment options for patients with naïve or recurrent follicular lymphoma are still controversial, ranging from a "watch and wait" policy to hematopoietic stem cell transplantation. More recently, the availability of rituximab has substantially changed follicular lymphoma therapeutic approaches to such an extent that R-Chemo is now the standard induction first-line treatment. This review provides a general overview of the state of the art in the management of follicular lymphoma and also, a brief description regarding the current prognostic tools available for treatment decisions.

Resumo em Inglês:

This study aims to critically review the literature in respect to craniofacial bone abnormalities and malocclusion in sickle cell anemia individuals. The Bireme and Pubmed electronic databases were searched using the following keywords: malocclusion, maxillofacial abnormalities, and Angle Class I, Class II and lass III malocclusions combined with sickle cell anemia. The search was limited to publications in English, Spanish or Portuguese with review articles and clinical cases being excluded from this study. Ten scientific publications were identified, of which three were not included as they were review articles. There was a consistent observation of orthodontic and orthopedic variations associated with sickle cell anemia, especially maxillary protrusions. However, convenience sampling, sometimes without any control group, and the lack of estimates of association and hypotheses testing undermined the possibility of causal inferences. It was concluded that despite the high frequency of craniofacial bone abnormalities and malocclusion among patients with sickle cell anemia, there is insufficient scientific proof that this disease causes malocclusion