ABSTRACT Background: The large diversity of red blood cell antigens favors, especially in multi-transfused patients, the occurrence of autoimmunization and alloimmunization with the risk of hemolytic transfusion reactions. Thus, this study aimed to determine the rates of alloimmunization and autoimmunization in these individuals, as well as the types of alloantibodies and their systems, clinical and epidemiological aspects and the frequency of autoimmunity in alloimmunized and non-alloimmunized patients. Methods: In a retrospective study, 153 multi-transfused patients from 2006 to 2014 were evaluated. Sixty-eight had onco-hematological diseases, 64 had hemoglobinopathies and 21 had chronic renal failure. Descriptive analyses were carried out with the proportions being compared using the chi-square test, with the significance level set at 5%. Results: The Rh system was the most frequently involved (53.11%) and anti-E and anti-K (Kell system) were the most prevalent alloantibodies (21.87% each). Autoantibodies were found in ten patients (6.54%) with the percentages of autoimmunization in alloimmunized and non-alloimmunized individuals being 29.16% and 2.32%, respectively (p = 0.0001). There was a significant difference between autoimmunization and the number of transfusions (16.21% in 6–10 vs. 5.26% <6 vs. 2.56% >10; p = 0.0203) and diseases (19.04% in chronic renal failure vs. 6.25% in hemoglobinopathies vs. 2.94% in onco-hematological diseases; p = 0.0329). Conclusion: The results show a strong correlation between alloimmunization and autoimmunization. Moreover, they reinforce the need for further studies on the clinical and epidemiological profile of multi-transfused patients in relation to alloimmunity and autoimmunity, especially the latter, for a better understanding of its etiopathogenesis and physiopathogenesis.

Abstract Introduction: The clinical manifestations of sickle cell disease are related to the polymerization of hemoglobin S. The chronic hemolysis caused by this condition often causes the formation of gallstones that can migrate and block the common bile duct leading to acute abdomen. Objective: This study aimed to evaluate the profile of patients with sickle cell disease and cholelithiasis. Methods: Patients with sickle cell disease were separated into groups according to the presence or absence of cholelithiasis. Socioepidemiological and clinical characteristics, such as gender, age, use of hydroxyurea and the presence of other hemoglobinopathies were researched in the medical records of patients. Results: A hundred and seven patients with sickle cell anemia were treated at the institution. Of these, 27 (25.2%) had cholelithiasis. The presence of cholelithiasis was higher in the 11–29 age group than in younger than 11 years and over 29 years. No association was found for the presence of cholelithiasis with gender, use of hydroxyurea or type of hemoglobinopathy (hemoglobin SS, hemoglobin SC or sickle beta-thalassemia). Sixteen of the patients had to be submitted to cholecystectomy with 14 of the surgeries being performed by laparoscopy. Complications were observed in three patients and one patient died for reasons unrelated to the surgery. Conclusion: A quarter of patients with sickle cell disease had gallstones, more commonly in the 11- to 29-year age range. Patients should be monitored from childhood to prevent cholelithiasis with preoperative, intra-operative and postoperative care being crucial to reduce the risk of complications in these patients.

RESUMO CONTEXTO E OBJETIVO: As hemoglobinopatias estão entre as alterações genéticas mais comuns e mundialmente difundidas, variando conforme a composição étnica e/ou geográfica. Nosso objetivo foi verificar a incidência de hemoglobinopatias por métodos de triagem laboratorial e sua associação com a etnia em 1.004 recém-nascidos. TIPO DE ESTUDO E LOCAL: Estudo transversal; realizado em um hospital público de referência do Triângulo Mineiro, Minas Gerais, Brasil. MÉTODOS: Avaliação qualitativa das hemoglobinas foi realizada por eletroforese em acetato de celulose: em pH alcalino para identificação do perfil hemoglobínico, em pH ácido para diferenciação das Hb S da Hb D e Hb C da Hb E, e outras que migrem em posições semelhantes em pH alcalino e em pH neutro para detecção de Hb Bart's, identificada na talassemia alfa (α-thal); e avaliação quantitativa das hemoglobinas pelo método de eluição após eletroforese. RESULTADOS: Houve predomínio da α-thal, com 105 (10,46%) casos, seguida da Hb S, com 61 casos (6,08% - 46 Hb AS, 2 Hb SS e 13 Hb S/α-thal), 9 casos (0,9%) de Hb AC e 6 (0,6%) sugestivos de beta-talassemia (β-thal). A incidência de hemoglobinopatias foi significantemente maior em afrodescendentes. CONCLUSÕES: Esses achados reforçam a importância do Programa Nacional de Triagem Neonatal para o diagnóstico das síndromes falciformes e hemoglobinopatias C, D, E e β-thal e robustecem os dados da literatura sobre a necessidade da pesquisa da α-thal em sangue de cordão (não identificada pelo Programa Nacional) e em pacientes com anemia microcítica não responsivos à ferroterapia.

ABSTRACT CONTEXT AND OBJECTIVE: Hemoglobinopathies are among the commonest and most widespread genetic disorders worldwide. Their prevalence varies according to ethnic composition and/or geographical region. The aim of this study was to investigate the presence of hemoglobinopathies and their association with ethnicity among 1,004 newborns, to confirm the guideline of the Brazilian National Neonatal Screening Program. DESIGN AND SETTING: Cross-sectional study conducted in a public referral hospital in the Triângulo Mineiro region, Minas Gerais, Brazil. METHODS: Qualitative assessment of hemoglobin was performed through electrophoresis on cellulose acetate: at alkaline pH to identify the hemoglobin (Hb) profile and at acid pH to differentiate Hb S from Hb D and Hb C from Hb E and others that migrate to similar positions at alkaline pH. Neutral pH was used to detect Hb Bart's identified in alpha thalassemia (α-thal). The elution method after electrophoresis was used to quantitatively assess hemoglobins. RESULTS: There was predominance of α-thal, with 105 cases (10.46%), followed by Hb S with 61 cases (6.08%, comprising 46 Hb AS, 2 Hb SS and 13 Hb S/α-thal), 9 cases (0.9%) of Hb AC and 6 cases (0.6%) suggestive of beta thalassemia (β-thal). The frequency of hemoglobinopathies was significantly higher among Afro-descendants. CONCLUSIONS: These findings corroborated of the National Neonatal Screening Program for diagnosing sickle cell disease and Hb C, Hb D, Hb E and β-thal hemoglobinopathies.

Background: Hemovigilance is an organized system of surveillance throughout the transfusion chain intended to evaluate information in order to prevent the appearance or recurrence of adverse reactions related to the use of blood products. Objective: The aims of this study were to assess the late reporting of incidents related to possible seroconversion in respect to age, marital status and ethnical background, annual variations in late reporting, the number of reports opened and closed, seroconversion of donors and transfusions of blood products within the window period. Methods: This retrospective, descriptive study used data on blood donations in the blood bank in Uberaba during the period from 2004 to 2011. Some socio-epidemiological characteristics of the donors and serology test results of donors and recipients were analyzed in respect to the late reporting of incidents related to possible seroconversion. The Chi-square test, odds ratio and a regression model were used for statistical analysis. Results: From 2004 to 2011, the blood bank in Uberaba collected 117,857 blood bags, 284 (0.24%) of which were investigated for late reported incidents. The profile of the donors was less than 29 years old, unmarried and non-Whites. Differences in age (p-value < 0.0001), marital status (p-value = 0.0002) and ethnical background (p-value < 0.0001) were found to be statistically significant. There was no statistical difference between men and women (0.24% and 0.23% respectively; p-value = 0.951). The number of late reported incidents increased until 2008 followed by a downward trend until 2011. There were twelve cases of seroconversion in subsequent donations (seven human immunodeficiency virus, four hepatitis B and one hepatitis C) with proven human immunodeficiency virus infection after screening of only one recipient. Conclusion: The twelve cases of seroconversion in donors with subsequent infection proven in one recipient underscores the importance of this tool to increase transfusion safety.

BACKGROUND: Sickle cell disease, the most common hereditary blood disease in the world, is the result of an atypical hemoglobin called S (Hb S) which, when homozygous (Hb SS) is the cause of sickle cell anemia. Changes of puberty, correlated with a delayed growth spurt, begin late in both male and female sickle cell anemia individuals with repercussions on sexuality and reproduction. The objectives of this exploratory and descriptive study were to characterize the development of sexuality in adults with sickle cell anemia by investigating the patient's perception of their sex life, as well as the information they had and needed on this subject. METHODS: Twenty male and female sickle cell anemia patients treated at the Hemocentro Regional de Uberaba (UFTM) with ages between 19 and 47 years old were enrolled. A socioeconomic questionnaire and a semi-structured interview on sexuality, reproduction and genetic counseling were applied. RESULTS: This study shows that the sickle cell anemia patients lacked information on sexuality especially about the risks of pregnancy and the possible inheritance of the disease by their children. Moreover, the sexual life of the patients was impaired due to pain as well as discrimination and negative feelings experienced in close relationships. CONCLUSION: The health care of sickle cell anemia patients should take into account not only the clinical aspects of the disease, but also psychosocial aspects by providing counseling on sexuality, reproduction and genetics, in order to give this population the possibility of a better quality of life.

INTRODUÇÃO: Estudo retrospectivo com o objetivo de avaliar a ocorrência de doações inaptas para doença de Chagas entre 1995 e 2009 no Hemocentro Regional de Uberaba (HRU), Brasil; verificar a tendência dessa inaptidão e descrever o perfil epidemiológico dos doadores. MÉTODOS: Estudo retrospectivo da inaptidão sorológica para doença de Chagas; análise estatística pelo teste do qui quadrado e odds ratio; estudo de tendência a partir do gráfico de dispersão e do coeficiente de correlação linear (r). RESULTADOS: No período em estudo foi encontrada uma soroprevalência de inaptidão para doença de Chagas de 0.2% com queda significativa de doações inaptas de 2001 a 2009. Dentre os soropositivos, observou-se predomínio significante na idade superior ou igual a 30 anos, e estado civil casado e outros. CONCLUSÕES: Os resultados demonstram taxa de ocorrência menor que as descritas na literatura, além de queda progressiva no decorrer dos 15 anos avaliados. Tais resultados são consequência do combate sistemático ao vetor a partir dos anos 70 e do progressivo e consistente aumento de doadores de retorno, resultando em queda do risco de contaminação via transfusão de sangue e na melhora da qualidade da hemoterapia praticada no HRU.

INTRODUCTION: A retrospective study was conducted to assess the occurrence of blood donations that were ineligible due to Chagas disease infection from 1995 to 2009 at the Uberaba Regional Blood Center (HRU), Brazil, verify the tendency of this ineligibility, and describe the epidemiologic profile of the donors. METHODS: Retrospective studies of serological ineligibility due to Chagas disease, statistical analysis by means of the chi-square test and odds ratio, study of the tendencies using a dispersion graph and the linear correlation coefficient (r) were performed. RESULTS: In the period under study, a 0.2% serum prevalence of ineligibility due to Chagas disease was found, with a significant drop in ineligible donations from 2001 to 2009. Among the serum positive-donors, there was a significant predominance among those aged 30 years or above and non-single individuals. CONCLUSIONS: The results show a rate of occurrence that is lower than that described in literature, as well as a progressive drop during the 15 years under assessment. Such results are a consequence of systematic combat of the vector since the 70s and the progressive and consistent increase of returning donors, resulting in a drop of the contamination risk factor by means of blood transfusion and in the improvement of the quality of hemotherapy practices in the HRU.

OBJETIVO: Vários aloanticorpos irregulares antieritrocitários, produzidos por sensibilizações a antígenos estranhos durante gestações ou transfusões, possuem importância clínica por provocarem hemólise no feto ou recém-nascido e/ou no receptor de sangue. Diante disso, fizemos uma análise prospectiva exclusiva de pacientes atendidos nos serviços de emergência clínica e cirúrgica, correlacionando as aloimunizações aos dados clínicos e epidemiológicos. MÉTODOS: Amostras sanguíneas de 143 pacientes, com pesquisa inicial de anticorpos negativa, foram coletadas até 15 meses após transfusões de concentrados de hemácias (CH), submetidas a Pesquisas de Anticorpos Irregulares (PAI) e, quando o resultado fosse positivo, à identificação e titulação seriada do(s) aloanticorpo(s). Para comparar proporções, foi empregado Teste Exato de Fisher e Odds Ratio. RESULTADOS: Dos pacientes estudados, 15 (10,49%) produziram aloanticorpos em um período de seis meses, com o título decaindo e negativando até 15 meses em 60% dos aloimunizados. Os aloanticorpos anti-K e os dirigidos contra antígenos do sistema Rh foram os mais frequente, o maior título foi de 1:32 (anti-K) e houve evidente correlação com o número de transfusões. CONCLUSÃO: Diante da alta ocorrência de aloanticorpos antieritrocitários de importância clínica em indivíduos transfundidos em serviço de emergência clínica e cirúrgica, propomos estender a fenotipagem e compatibilização pré-transfusional para os antígenos C, c, E, e (sistema Rh) e K (sistema Kell) para todos os pacientes com cirurgias programadas e/ou com eventos clínicos agudos que não necessitem de transfusões emergenciais.

BACKGROUND: Several irregular red blood cell alloantibodies, produced by alloimmunization of antigens in transfusions or pregnancies, have clinical importance because they cause hemolysis in the fetus and newborn and in transfused patients. OBJECTIVE: a prospective analysis of patients treated by the surgical and clinical emergency services of Hospital de Clínicas of the Universidade Federal do Triângulo Mineiro (HC/UFTM), Brazil was performed to correlate alloimmunization to clinical and epidemiological data. METHODS: Blood samples of 143 patients with initial negative antibody screening were collected at intervals for up to 15 months after the transfusion of packed red blood cells. Samples were submitted to irregular antibody testing and, when positive, to the identification and serial titration of alloantibodies. The Fisher Exact test and Odds Ratio were employed to compare proportions. RESULTS: Fifteen (10.49%) patients produced antibodies within six months of transfusion. However, for 60% of these individuals, the titers decreased and disappeared by 15 months after transfusion. Anti-K antibodies and alloantibodies against antigens of the Rh system were the most common; the highest titer was 1:32 (anti-K). There was an evident correlation with the number of transfusions. CONCLUSIONS: Given the high incidence of clinically important red blood cell alloantibodies in patients transfused in surgical and clinical emergency services, we suggest that phenotyping and pre-transfusion compatibilization for C, c, E, e (Rh system) and K (Kell system) antigens should be extended to all patients with programmed surgeries or acute clinical events that do not need emergency transfusions.

INTRODUCÃO: Estudo retrospectivo com o objetivo de avaliar a prevalência e fatores associados à soropositividade para o HTLV-1/2, no período de 1995 a 2008, no Hemocentro Regional de Uberaba e descrever os doadores soropositivos quanto ao gênero, idade, estado civil, cor de pele e procedência. MÉTODOS: Foram realizados análise estatística descritiva, testes qui-quadrado e odds ratio para comparação de proporções e gráfico de dispersão com coeficiente de correlação linear. RESULTADOS: Dentre x doadores testados, foi encontrada a prevalência de sorologia positiva para o HTLV de 0,02% e indeterminada de 0,09%. Houve uma redução significativa da sorologia positiva para HTLV, no período de 2002 a 2008, em comparação ao período de 1995 a 2001. Dentre os soropositivos, observou predomínio significante no gênero feminino. CONCLUSÕES: Imputamos a queda gradativa de soropositividade no período à exclusão permanente dos doadores de repetição soropositivos e ao aprimoramento dos métodos de triagem clínica e dos testes sorológicos ao longo dos anos com reflexos positivos na segurança transfusional.

INTRODUCTION: A retrospective study was conducted in order to assess the prevalence and factors associated with seropositivity for HTLV-1/2 between 1995 and 2008 in Uberaba Regional Blood Center, and to describe the seropositive blood donors in relation to gender, age, marital status, skin color and origin. METHODS: Descriptive statistical analysis, chi-square tests and odds ratios were produced to compare proportions, along with scatter charts with linear correlation coefficients. RESULTS: Among the donors tested, the prevalence of seropositivity for HTLV was found to be 0.02%, with indeterminate results in 0.09%. There was a significant reduction in seropositivity for HTLV between 2002 and 2008, compared with the period from 1995 to 2001. Among the seropositive individuals, females were significantly predominant. CONCLUSIONS: The gradual decrease in seropositivity over this period was attributed to the permanent exclusion of seropositive repeat donors and improvement in the clinical screening methods and serological tests over the years, with a positive impact on transfusion safety.

A alta morbimortalidade referida na doença falciforme (DF) levou-nos a estudar o perfil epidemiológico e respectivas intercorrências clínicas dos pacientes atendidos no Hemocentro Regional (HR) e Hospital de Clínicas da Universidade (HC-U), de 1998 a 2007. Estudo retrospectivo de 151 pacientes, avaliados quanto a: idade, gênero, cor da pele, procedência, diagnóstico, causa de atendimento no HR, causa e tempo de internação no HC-U e a causa e a idade em caso de óbito. Foi realizada análise estatística descritiva simples. A média de idade foi de 17,7 anos, 52,4% eram do gênero feminino, 58,2% procediam da cidade de Uberaba e em 92,2% dos prontuários não foram encontrados relatos sobre a cor da pele. A anemia falciforme representou 82,5% dos casos. Dos 910 atendimentos no HR e 589 internações no HC-U, a crise dolorosa afebril foi a causa mais frequente em ambas as instituições (61,9% e 25,3%, respectivamente). A idade média dos 11 óbitos foi de 33,5 anos, sendo apenas um em menor de 10 anos e a falência de múltiplos órgãos a causa mais frequente. O perfil epidemiológico mostra predomínio de crianças e adultos jovens, sexo feminino e genótipo SS. As taxas de internação no HC-U, de atendimento no HR e a baixa média de idade ao óbito confirmam a alta morbidade e mortalidade da DF. Contudo, o grande número de crianças sem intercorrências e/ou internações reflete a eficácia das medidas preventivas propiciadas pelo diagnóstico precoce implantado nos últimos 10 anos.

The high morbidity-mortality rates in sickle cell disease (SCD) led us to study the epidemiological profile and respective clinical complications of patients seen at a Regional Blood Center (HR) and a University Clinical Hospital (HC-U) between 1998 and 2007. In a retrospective study, 151 patients were evaluated regarding age, gender, skin color, origin, diagnosis, reason of visit to HR or reason and length of stay in the HC-U, and cause and age in cases of death. Simple descriptive statistical analysis was performed. The mean age was 17.7 years, 52.4% of the patients were female, 58.2% were from the city and no information regarding skin color was available in 92.2% of the records. Sickle cell anemia was present in 82.5% of cases. The most frequent reason for the 910 visits to HR and 589 hospitalizations in HC-U were afebrile pain episodes (61.9% and 25.3%, respectively). The mean age of the 11 patients who died was 33.5 years with one patient being younger than 10 years old, and multiple organ failure was the most frequent cause. The epidemiological profile shows a predominance of children and young adults, women, and the SS genotype. The rates of hospitalization in HC-U and of visits to HR and the low mean age at death confirm the high morbidity and mortality related to SCD. However, a large number of children do not present with complications or require hospitalization which reflects the efficacy of preventive measures provided by the early diagnosis implemented over the last 10 years.

O objetivo deste estudo foi traçar o perfil sorológico para a hepatite C nos doadores de sangue do Hemocentro Regional de Uberaba/Fundação Hemominas nos últimos 14 anos, comparando a sua ocorrência em doadores iniciais e de retorno e analisando as características epidemiológicas daqueles com ELISA anti-HCV (terceira e quarta geração) positivo e indeterminado, a partir dos históricos dos doadores nos arquivos do Hemocentro Regional de Uberaba. A inaptidão sorológica encontrada foi de 0,3%, com maior ocorrência no grupo de doadores iniciais. Não encontramos diferenças significativas quanto à idade, cor da pele, estado civil e local de residência entre os aptos e os inaptos, porém a ocorrência de sorologia positiva foi maior nos homens. A menor prevalência de inaptidão para hepatite C (0,3%) no Hemocentro Regional de Uberaba, em relação à maioria dos hemocentros do país, é provavelmente devido ao grande (83,3%) número de doadores de repetição, reforçando assim a importância da fidelização do doador para o aumento da segurança transfusional.

The aim of the present study was to outline the serological profile of hepatitis C among blood donors seen at the Uberaba Regional Blood Center, Hemominas Foundation, over the last 14 years. The frequency of hepatitis C was compared between first-time and repeat donors and the epidemiological characteristics of those with positive and indeterminate ELISA anti-HCV (third and fourth generation) were analyzed based on the donor histories kept in the archives of the Uberaba Regional Blood Center. The serological ineligibility rate was 0.3%, with higher prevalence in the group of first-time donors. We did not find any significant differences regarding age, skin color, marital status or place of residence between eligible and ineligible donors; however, the frequency of positive serology was higher among men. The lower (0.3%) rate of ineligibility due to hepatitis C that was observed at the Uberaba Regional Blood Center, in relation to most Brazilian blood centers, is probably due to the large number of repeat donors (83.3%). This reinforces the importance of achieving donor commitment for increasing transfusion safety.