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Background: Immune tolerance induction (ITI) is the treatment of choice to eradicate neutralizing anti-factor (F)VIII alloantibodies (inhibitors) in people with inherited hemophilia A. However, it is not successful in 10% to 40% of the cases. The biological mechanisms and biomarkers associated with ITI outcome are largely unknown. Objectives: The aim of this study was to investigate the association of plasma cytokines (interferon-γ, tumor necrosis factor, interleukin [IL]-2, IL-4, IL-5, IL-6, IL-10, and IL-17A), chemokines (IL-8/CXCL8, RANTES/CCL5, MIG/CXCL9, MCP-1/CCL2, and IP-10/CXCL10), and anti-FVIII immunoglobulin (Ig) G total, IgG1, and IgG4 with ITI outcome. Methods: In this cross-sectional analysis of the Brazilian Immune Tolerance Study, we assessed plasma levels of anti-FVIII IgGs using an enzyme-linked immunosorbent assay with plasma-derived FVIII and recombinant FVIII as target antigens, immobilized in microplates. Results: We assayed 98 plasma samples of moderately severe and severe (FVIII activity, <2%) people with hemophilia A after completion of a first ITI course. Levels of anti-recombinant FVIII IgG total and IgG4 were higher in people with hemophilia A who failed ITI (IgG total optical density [OD], 0.37; IQR, 0.15-0.73; IgG4 OD, 2.19; IQR, 0.80-2.52) than in those who had partial (IgG total OD, 0.03; IQR, 0.00-0.14; IgG4 OD, 0.39; IQR, 0.09-1.11; P < .0001 for both) or complete success (IgG total OD, 0.04; IQR, 0.00-0.07; IgG4 OD, 0.07; IQR, 0.06-0.40; P < .0001 for both). Plasma cytokines, chemokines, and anti-FVIII IgG1 were not associated with ITI outcome. Conclusion: Our results show that high levels of plasma anti-FVIII IgG4 and IgG total are associated with ITI failure.

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ABSTRACT Introduction: The Glanzmann Thrombasthenia (GT) and Bernard-Soulier Syndrome (BSS) are rare hereditary disorders of platelet function. Their treatment often requires platelet transfusion, which can lead to the development of alloantibodies. Objective: In this study, we aim to develop a strategy for alloantibody detection and to describe the frequency of alloimmunization in a patient population from a single center in southeastern Brazil. Methods: Samples from patients with GT or BSS were tested using the Platelet Immunofluorescence Test (PIFT). If a positive result was obtained, a confirmatory step using the Monoclonal Antibody Immobilization of Platelet Antigens (MAIPA) and Luminex bead-based platelet assay (PAKLx) was executed. Main results: Among 11 patients with GT, we detected the presence of alloantibodies in 5 using PIFT, with confirmation through MAIPA and PAKLx in 2 (1 anti-HLA and 1 anti-HPA), resulting in a frequency of 18.1%. Among 4 patients with BSS, PIFT was positive in 3, with confirmation by MAIPA and PAKLx in 1 (anti-HLA), showing a frequency of 25%. The two patients with anti-HLA antibodies exhibited a panel reactive antibody (PRA-HLA) testing greater than 97%. Conclusion: Our study highlights the importance of identifying platelet alloimmunization in this patient population. The proposed algorithm for platelet alloantibodies detection allows resource optimization.

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INTRODUCTION: The Glanzmann Thrombasthenia (GT) and Bernard-Soulier Syndrome (BSS) are rare hereditary disorders of platelet function. Their treatment often requires platelet transfusion, which can lead to the development of alloantibodies. OBJECTIVE: In this study, we aim to develop a strategy for alloantibody detection and to describe the frequency of alloimmunization in a patient population from a single center in southeastern Brazil. METHODS: Samples from patients with GT or BSS were tested using the Platelet Immunofluorescence Test (PIFT). If a positive result was obtained, a confirmatory step using the Monoclonal Antibody Immobilization of Platelet Antigens (MAIPA) and Luminex bead-based platelet assay (PAKLx) was executed. MAIN RESULTS: Among 11 patients with GT, we detected the presence of alloantibodies in 5 using PIFT, with confirmation through MAIPA and PAKLx in 2 (1 anti-HLA and 1 anti-HPA), resulting in a frequency of 18.1%. Among 4 patients with BSS, PIFT was positive in 3, with confirmation by MAIPA and PAKLx in 1 (anti-HLA), showing a frequency of 25%. The two patients with anti-HLA antibodies exhibited a panel reactive antibody (PRA-HLA) testing greater than 97%. CONCLUSION: Our study highlights the importance of identifying platelet alloimmunization in this patient population. The proposed algorithm for platelet alloantibodies detection allows resource optimization.

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A severe complication of allogeneic hematopoietic stem cell transplantation (HSCT) is graft failure (GF). Among others, donor-specific anti-HLA antibodies (DSA) are associated with graft rejection after allogeneic or haploidentical transplantation in adults. Knowledge of DSA and pediatric recipients is limited. Hence, we aimed to generate more information about the presence of DSA (pre- and post-HSCT) and the clinical outcomes (graft rejection and poor function) in children. We identified DSA in 27% of the patients. We observed a higher frequency (50%) of DSA-bearing patients with a benign disease diagnosis than those diagnosed with leukemia (16.66%). We observed graft rejection in one patient (with DSA against two alleles of HLA class I molecules) and poor function in three recipients during the first 30 days after HSCT in the absence of DSA. The presence of donor and nondonor HLA-specific antibodies decreased substantially after transplantation. After the transplant, we identified two patients with DSA specific for HLA class I molecules (independent of clinical relevance), and four recipients showed PGF in the absence of DSA. We were unable to establish any association between the presence of DSA and a clinical outcome: graft failure or prevalence of viral infection.

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ABSTRACT: This study aimed to determine the erythrocyte phenotypes of the feline AB system and to check the presence of antigens other than those present in the feline AB system in domestic cats from Ilhéus-Itabuna microregion, Bahia, Brazil. Three-hundred feline blood samples were collected at the Veterinary Hospital of the Universidade Estadual de Santa Cruz (UESC) and in home visits to perform blood phenotyping using the tube-method testing. The reverse phenotyping was made between cats that tested phenotype B with blood samples of cats that tested phenotype A to confirm the blood phenotype B. The cross-tested among cats with phenotype A was made in order to verify the presence of different antigens of AB system in this blood phenotype. The results underwent macroscopic and microscopic analyses. Among the 300 animals tested, regarding breed, 290 were mixed-breed cats and among the remaining ten, five were Persians, four Siamese, and one Angora. 297 (99%) presented with phenotype A (including all the breeding cats) and three (1%) with phenotype B, and all this cats were mixed-breed cats. None (0%) of the cats showed the phenotype AB. All phenotype B bloods reacted to reverse phenotyping with phenotype A, confirming the phenotype B of these cats. All phenotype A bloods were compatible among each other, so no further erythrocyte antigens were detected through this test. The mother of one of the phenotype B cats was identified and had phenotype A, demonstrating phenotype A parents with phenotype B offspring. This finding indicates heterozygosis in the studied population. This data enable to conclude that the studied population presented different erythrocyte phenotypes, subsequently highlighting the importance of conducting phenotype analyses in these animals before performing blood transfusion to avoid serious hemolytic complications associated with incompatibility.

RESUMO: O objetivo deste estudo foi determinar a frequência dos fenótipos eritrocitários do sistema AB felino e verificar a presença de outros antígenos, não pertencentes ao sistema AB felino, em gatos domésticos das cidades de Ilhéus e Itabuna, Bahia, Brasil. Amostras de sangue de 300 gatos foram coletadas no Hospital Veterinário da Universidade Estadual de Santa Cruz (UESC) e em visitas domiciliares para realizar a fenotipagem sanguínea usando o método de tubo. A fenotipagem reversa foi realizada em gatos que testaram o fenótipo B com amostras que testaram o fenótipo A, para confirmação do fenótipo sanguíneo. O teste cruzado foi realizado entre gatos do fenótipo A, para pesquisar a presença de diferentes antígenos do sistema AB dentro desse fenótipo sanguíneo. Os resultados foram submetidos a análises macroscópicas e microscópicas. Dos 300 animais testados, 110 eram machos e 190 fêmeas, e suas idades variaram de cinco meses à 15 anos. Sobre as raças, 290 eram gatos sem raça definida e dos 10 restantes, cinco eram Persas, quatro eram Siameses e um Angorá. 297 (99%) apresentaram fenótipo A (incluindo todos os gatos de raça) e três (1%) tiveram fenótipo B, sendo todos esses gatos sem raça definida. Nenhum (0%) dos gatos apresentou fenótipo AB. Todos os sangues com fenótipo B reagiram na fenotipagem reversa com o fenótipo A, confirmando o fenótipo B desses gatos. Todos os sangues com fenótipo A foram compatíveis entre si, portanto nenhum antígeno eritrocitário adicional foi detectado através deste teste. A genitora de um dos gatos com fenótipo B, foi encontrada e a mesma possuía fenótipo A, demonstrando pais com fenótipo A e cria com fenótipo B. Esse achado indica heterozigose na população estudada. Esses dados levam à conclusão de que diferentes fenótipos eritrocitários estão presentes na população estudada e destacam a importância da realização de testes fenotípicos nesses animais antes dos procedimentos de transfusão, a fim de evitar complicações hemolíticas graves decorrentes do envolvimento de animais incompatíveis.

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La hemofilia A es una enfermedad hereditaria ligada al cromosoma X, causada por mutaciones en el gen F8 del factor VIII de la coagulación. Se considera una enfermedad huérfana, ya que su prevalencia es baja, de 26,6 por cada 100.000 nacidos vivos de sexo masculino. Los pacientes con hemofilia A tienen fases de inicio y amplificación de la coagulación relativamente normales y son capaces de formar el tapón plaquetario inicial en el lugar de la hemorragia, pero debido a la deficiencia del factor VIII, son incapaces de generar una cantidad de trombina en la superficie de las plaquetas, que sea suficiente para estabilizar el coágulo de fibrina. En un paciente masculino con hemorragias inusuales debe descartarse un trastorno de coagulación tipo hemofilia A, y se debe solicitar un recuento de plaquetas y un tiempo de protrombina (TP), los cuales usualmente son normales, y un tiempo de tromboplastina parcial activado (TPT) que se presenta prolongado. Para el diagnóstico diferencial con otras coagulopatías se realiza la medición de factores de coagulación, y pruebas de corrección cuando existe la sospecha de un inhibidor o de una hemofilia adquirida. Los pacientes afectados pueden presentar formas leves, moderadas o severas de la enfermedad, según el nivel plasmático del factor. En Colombia y en el mundo, la hemofilia fue reconocida como una enfermedad huérfana que representa un problema de salud pública, debido a su proceso de atención altamente especializado, que incrementa los costos asociados con la asistencia sanitaria, y afecta la calidad de vida de los pacientes y de aquellos que los rodean, además de que representa un reto diagnóstico que requiere constante actualización, para que pueda ser tratada de manera efectiva

Hemophilia A is an X-linked inherited disease caused by mutations in the coagulation factor VIII F8 gene. It is considered a rare disease, as its prevalence is 26.6 per 100,000 live male births. Patients with hemophilia A have a relatively normal coagulation onset and amplification phases, and are able to form the initial platelet plug at the site of hemorrhage; but due to factor VIII deficiency, they are unable to generate a sufficient amount of thrombin on the platelet surface to stabilize the fibrin clot. In a male patient with unusual bleeding, a hemophilia A-type coagulation disorder should be ruled out, and blood tests such as a platelet count and prothrombin time (PT), which are usually normal, and an activated partial thromboplastin time (APTT), which is prolonged, should be requested immediately. For differential diagnosis with other coagulopathies, measurement of coagulation factors and correction tests are performed when there is suspicion of an inhibitor or acquired hemophilia. Affected patients may present mild, moderate or severe forms of the disease, depending on the plasma level of the factor. In Colombia and worldwide, hemophilia was recognized as a rare disease that represents a public health problem due to its highly specialized care, which increases the costs associated with health care, and affects the quality of life of patients and those around them, as well as representing a diagnostic challenge that requires constant updating, so that it can be treated effectively

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This study aimed to determine the erythrocyte phenotypes of the feline AB system and to check the presence of antigens other than those present in the feline AB system in domestic cats from Ilhéus-Itabuna microregion, Bahia, Brazil. Three-hundred feline blood samples were collected at the Veterinary Hospital of the "Universidade Estadual de Santa Cruz" (UESC) and in home visits to perform blood phenotyping using the tube-method testing. The reverse phenotyping was made between cats that tested phenotype B with blood samples of cats that tested phenotype A to confirm the blood phenotype B. The cross-tested among cats with phenotype A was made in order to verify the presence of different antigens of AB system in this blood phenotype. The results underwent macroscopic and microscopic analyses. Among the 300 animals tested, regarding breed, 290 were mixed-breed cats and among the remaining ten, five were Persians, four Siamese, and one Angora. 297 (99%) presented with phenotype A (including all the breeding cats) and three (1%) with phenotype B, and all this cats were mixed-breed cats. None (0%) of the cats showed the phenotype AB. All phenotype B bloods reacted to reverse phenotyping with phenotype A, confirming the phenotype B of these cats. All phenotype A bloods were compatible among each other, so no further erythrocyte antigens were detected through this test. The mother of one of the phenotype B cats was identified and had phenotype A, demonstrating phenotype A parents with phenotype B offspring. This finding indicates heterozygosis in the studied population. This data enable to conclude that the studied population presented different erythrocyte phenotypes, subsequently highlighting the importance of conducting phenotype analyses in these animals before performing blood transfusion to avoid serious hemolytic complications associated with incompatibility.(AU)

O objetivo deste estudo foi determinar a frequência dos fenótipos eritrocitários do sistema AB felino e verificar a presença de outros antígenos, não pertencentes ao sistema AB felino, em gatos domésticos das cidades de Ilhéus e Itabuna, Bahia, Brasil. Amostras de sangue de 300 gatos foram coletadas no Hospital Veterinário da Universidade Estadual de Santa Cruz (UESC) e em visitas domiciliares para realizar a fenotipagem sanguínea usando o método de tubo. A fenotipagem reversa foi realizada em gatos que testaram o fenótipo B com amostras que testaram o fenótipo A, para confirmação do fenótipo sanguíneo. O teste cruzado foi realizado entre gatos do fenótipo A, para pesquisar a presença de diferentes antígenos do sistema AB dentro desse fenótipo sanguíneo. Os resultados foram submetidos a análises macroscópicas e microscópicas. Dos 300 animais testados, 110 eram machos e 190 fêmeas, e suas idades variaram de cinco meses à 15 anos. Sobre as raças, 290 eram gatos sem raça definida e dos 10 restantes, cinco eram Persas, quatro eram Siameses e um Angorá. 297 (99%) apresentaram fenótipo A (incluindo todos os gatos de raça) e três (1%) tiveram fenótipo B, sendo todos esses gatos sem raça definida. Nenhum (0%) dos gatos apresentou fenótipo AB. Todos os sangues com fenótipo B reagiram na fenotipagem reversa com o fenótipo A, confirmando o fenótipo B desses gatos. Todos os sangues com fenótipo A foram compatíveis entre si, portanto nenhum antígeno eritrocitário adicional foi detectado através deste teste. A genitora de um dos gatos com fenótipo B, foi encontrada e a mesma possuía fenótipo A, demonstrando pais com fenótipo A e cria com fenótipo B. Esse achado indica heterozigose na população estudada. Esses dados levam à conclusão de que diferentes fenótipos eritrocitários estão presentes na população estudada e destacam a importância da realização de testes fenotípicos nesses animais antes dos procedimentos de transfusão, a fim de evitar complicações hemolíticas graves decorrentes do envolvimento de animais incompatíveis.(AU)

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This study aimed to determine the erythrocyte phenotypes of the feline AB system and to check the presence of antigens other than those present in the feline AB system in domestic cats from Ilhéus-Itabuna microregion, Bahia, Brazil. Three-hundred feline blood samples were collected at the Veterinary Hospital of the "Universidade Estadual de Santa Cruz" (UESC) and in home visits to perform blood phenotyping using the tube-method testing. The reverse phenotyping was made between cats that tested phenotype B with blood samples of cats that tested phenotype A to confirm the blood phenotype B. The cross-tested among cats with phenotype A was made in order to verify the presence of different antigens of AB system in this blood phenotype. The results underwent macroscopic and microscopic analyses. Among the 300 animals tested, regarding breed, 290 were mixed-breed cats and among the remaining ten, five were Persians, four Siamese, and one Angora. 297 (99%) presented with phenotype A (including all the breeding cats) and three (1%) with phenotype B, and all this cats were mixed-breed cats. None (0%) of the cats showed the phenotype AB. All phenotype B bloods reacted to reverse phenotyping with phenotype A, confirming the phenotype B of these cats. All phenotype A bloods were compatible among each other, so no further erythrocyte antigens were detected through this test. The mother of one of the phenotype B cats was identified and had phenotype A, demonstrating phenotype A parents with phenotype B offspring. This finding indicates heterozygosis in the studied population. This data enable to conclude that the studied population presented different erythrocyte phenotypes, subsequently highlighting the importance of conducting phenotype analyses in these animals before performing blood transfusion to avoid serious hemolytic complications associated with incompatibility.(AU)

O objetivo deste estudo foi determinar a frequência dos fenótipos eritrocitários do sistema AB felino e verificar a presença de outros antígenos, não pertencentes ao sistema AB felino, em gatos domésticos das cidades de Ilhéus e Itabuna, Bahia, Brasil. Amostras de sangue de 300 gatos foram coletadas no Hospital Veterinário da Universidade Estadual de Santa Cruz (UESC) e em visitas domiciliares para realizar a fenotipagem sanguínea usando o método de tubo. A fenotipagem reversa foi realizada em gatos que testaram o fenótipo B com amostras que testaram o fenótipo A, para confirmação do fenótipo sanguíneo. O teste cruzado foi realizado entre gatos do fenótipo A, para pesquisar a presença de diferentes antígenos do sistema AB dentro desse fenótipo sanguíneo. Os resultados foram submetidos a análises macroscópicas e microscópicas. Dos 300 animais testados, 110 eram machos e 190 fêmeas, e suas idades variaram de cinco meses à 15 anos. Sobre as raças, 290 eram gatos sem raça definida e dos 10 restantes, cinco eram Persas, quatro eram Siameses e um Angorá. 297 (99%) apresentaram fenótipo A (incluindo todos os gatos de raça) e três (1%) tiveram fenótipo B, sendo todos esses gatos sem raça definida. Nenhum (0%) dos gatos apresentou fenótipo AB. Todos os sangues com fenótipo B reagiram na fenotipagem reversa com o fenótipo A, confirmando o fenótipo B desses gatos. Todos os sangues com fenótipo A foram compatíveis entre si, portanto nenhum antígeno eritrocitário adicional foi detectado através deste teste. A genitora de um dos gatos com fenótipo B, foi encontrada e a mesma possuía fenótipo A, demonstrando pais com fenótipo A e cria com fenótipo B. Esse achado indica heterozigose na população estudada. Esses dados levam à conclusão de que diferentes fenótipos eritrocitários estão presentes na população estudada e destacam a importância da realização de testes fenotípicos nesses animais antes dos procedimentos de transfusão, a fim de evitar complicações hemolíticas graves decorrentes do envolvimento de animais incompatíveis.(AU)

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Quando um indivíduo é exposto a antígenos eritrocitários não próprios, ocorre uma resposta imunológica, que leva à produção de anticorpos irregulares voltados contra esses antígenos. Esse processo é conhecido como aloimunização eritrocitária e acontece em decorrência de transfusões de sangue ou gestações incompatíveis. Na medicina transfusional a pesquisa de anticorpos irregulares é fundamental, pois a falha na detecção de um aloanticorpo pode provocar reações transfusionais, aloimunizações, anemias hemolíticas autoimunes e doença hemolítica perinatal. Este estudo tem por objetivo analisar a frequência de anticorpos irregulares de pacientes atendidos no Hemocentro Regional de Francisco Beltrão, Paraná, no ano de 2017. Os dados foram coletados a partir da revisão de registros em arquivos do Laboratório de Imunohematologia do Hemonúcleo. Foram avaliados dados de 49 protocolos de pacientes que apresentaram dificuldades transfusionais no ano de 2017. Dentre os pesquisados, 37 pacientes (75,5%) apresentaram anticorpos irregulares. Dentre os anticorpos anti-eritrocitários observados neste estudo, evidenciou-se a presença de doze pacientes com anti-D (27,2%), seis pacientes com anti-K (13,6%), quatro pacientes com anti-C (9,0%) e em seis pacientes (13,6%) foi observada a presença de autoanticorpos. Este estudo indica que, nos pacientes transfundidos, os anticorpos mais frequentes foram os aloanticorpos Anti-D do Sistema Rh, provavelmente devido ao seu alto grau de imunogenicidade. A prevalência desses anticorpos é semelhante a vários estudos encontrados na literatura.

When an individual is exposed to not-self red blood cell antigens, an immune response occurs, which leads to the production of irregular antibodies directed against these antigens. This process is known as erythrocyte alloimmunization and occurs as a result of blood transfusions or incompatible pregnancies. In transfusion medicine, the search for irregular antibodies is essential, since failure to detect an alloantibody can cause transfusion reactions, alloimmunizations, autoimmune hemolytic anemias, and perinatal hemolytic disease. This study aims at analyzing the frequency of irregular antibodies of patients seen at the Regional Blood Center of Francisco Beltrão, Paraná, in 2017. The data were collected from the review of records in files of the Immunohematology Laboratory of Hemonúcleo. Data from 49 protocols of patients who had transfusion difficulties in 2017 were evaluated. Among those surveyed, 37 patients (75.5%) had irregular antibodies. Among the anti-erythrocyte antibodies observed in this study, the presence of twelve patients with anti-D (27.2%), six patients with anti-K (13.6%), four patients with anti-C (9.0 %), and in six patients (13.6%) with the presence of autoantibodies were observed. This study indicates that, in transfused patients, the most frequent antibodies were the Rh System Anti-D alloantibodies, probably due to their high degree of immunogenicity. The prevalence of these antibodies is similar to several studies found in the literature.

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INTRODUCTION: Alloimmunization is caused by exposure to erythrocytes from a donor that expresses blood group antigens other than those of the recipient and is related to processes that alter the balance of the immune system. Knowing the pathophysiology of alloimmunization process is essential to understand clinical complications associated with this process. PATIENTS AND METHODS: From October 2016 to April 2017, irregular antibody screening was performed in 1,434 polytransfused (compatible with the ABO and D system) patients by means of agglutination techniques using erythrocytes of a known phenotype of 44 patients with a positive alloantibody screening. Non-alloimmunized (control) subjects were matched for age, gender, pathology, and treatment group with alloimmunized patients. The subsets of B, T, and Treg lymphocytes were determined by flow cytometry. RESULTS: The results of screening for alloantibodies in patients by specificity of antibodies were as follows: nonspecific (30%), followed by anti-Dia (13%), anti-e (9%), anti-S (9%), anti-I (7%), anti-K (7%), and anti-P (7%). A lower percentage of CD4+ T lymphocytes and an increase of CD8+ T lymphocytes were observed in alloimmunized patients, as well as a low CD4/CD8 ratio (0.7 vs. 1.6, p = 0.003), a higher percentage of B lymphocytes versus the control group (30 vs. 20%, p = 0.003), and a decrease of Treg CD4+ lymphocytes versus the control group (3 vs. 12 cells/µL, p = 0.043). These observations suggest that alloimmunized patients have important alterations in the number of some lymphocyte subsets that can be translated into clinical immune dysregulation. CONCLUSION: A decreased CD4/CD8 ratio, increased B lymphocytes, and Treg lymphocyte deficiency are the most significant changes observed in alloimmunized patients.

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Introduction: To analyze the prevalence of alloantibodies in multiply transfused patients. Methods: This study was a retrospective, exploratory and descriptive study with a quantitative approach. The study sample comprised 185 patients transfused at a referral service in the city of Passo Fundo, Rio Grande do Sul, from January 2016 to February 2018. Results: Overall, the antibodies identified were as follows: anti-E in 47 patients (18%), anti-D and anti-K in 28 patients each (11%), anti-C in 21 patients (8.1%), and inconclusive antibody results in 23 patients (8.9%). Females were a majority (55.7%), mean age was 48.8 years and mean quantity of blood transfused was 7.2 bags. Cardiovascular disorders were the most common comorbidities, in 39 patients (21.2%), followed by oncological disorders, in 38 patients (18.4%). Conclusion: Alloimmunization is an important and frequent clinical condition that increases the risk of hemolytic reactions and is associated with significant patient morbidity and mortality. (AU)

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BACKGROUND: In Colombia, hemophilia is considered a high-cost disease, and hemophilia A with high-titer inhibitors may be responsible for a significant economic pressure on the Colombian health system. OBJECTIVES: To estimate the direct cost of care for patients with hemophilia A with high-titer inhibitors in Colombia, from the perspective of the health system. METHODS: A cost-of-illness study was carried out using standard case methodology, which was designed based on literature review and validation by expert consensus. Scenarios were established for adults and children, including cases of prophylaxis, immune tolerance induction, bleeding, and surgery. The frequencies were taken from the official report for Colombia, issued by the High-Cost Account 2017 (reported 2018). The prices were obtained from the list of regulated medicines in the country. The cost estimate is presented with a range of values by weight (between 10 kg and 90 kg). RESULTS: The total estimated cost per year for Colombia was US $44 905 252 (between US $32 260 497 and US $58 202 393). The average cost per year calculated for a patient was US $498 947 (between US $358 450 and US $646 693). A total of 99.8% of the estimated cost was directly related to the cost of the coagulation factors and bypassing agents. CONCLUSIONS: Hemophilia A with high-titer inhibitors is a disease that generates significant pressure on the Colombian health system, mainly linked to the cost of factors and bypassing agents.

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Introducción: Los anticuerpos irregulares corresponden a aquellos distintos a los anticuerpos naturales anti-A o anti-B, los cuales pueden aparecer en respuesta a la exposición a un antígeno eritrocitario extraño (transfusión o trasplante) o por incompatibilidad materno-fetal. Objetivo: Caracterizar a los donantes con rastreo de anticuerpos irregulares positivo de un banco de sangre de Montería, Colombia, durante el periodo 2012-2015. Métodos: Estudio transversal y retrospectivo, con fuente de información secundaria, basada en los resultados del rastreo de anticuerpos en los donantes de un banco de sangre de Montería, Colombia, entre los años 2012 y 2015. La población estuvo conformada por todos los donantes voluntarios registrados en el tiempo del estudio (35 248 donantes), a quienes se les realizó rastreo de anticuerpos. Como muestra, se seleccionaron todos los casos que tuvieron resultados positivos (71 donantes). Los datos fueron organizados en tablas y analizados en el software SPSS 21.0, Microsoft Excel y en Epidat versión 3.1. Resultados: El 0,2 por ciento de la población presentó un rastreo de anticuerpos positivo con un intervalo de confianza entre 0,15 y 0,25 por ciento. Los anticuerpos irregulares fueron más frecuentes en los hombres y en donantes O Rh positivo. Se encontraron Ac irregulares con 13 especificidades diferentes, con predomino de anti-M, anti-Lea, anti-D y anti-E y porcentajes respectivos de 27,78 por ciento, 20,83 por ciento, 9,72 por ciento y 8,33 por ciento. El 50 por ciento de los donantes tenía 30,5 años o menos, el 49,3 por ciento había donado previamente y el 9,9 por ciento recibió al menos una transfusión en algún momento de su vida. Conclusión: La frecuencia de donantes con rastreo de anticuerpos irregulares positivo fue baja, el sexo masculino presentó mayor porcentaje, se detectó principalmente en el grupo sanguíneo O y dentro de los anticuerpos irregulares, anti-M presentó una mayor frecuencia(AU)

Introduction: Irregular antibodies correspond to those other than natural anti-A or anti-B antibodies, which may appear in response to exposure to a foreign erythrocyte antigen (transfusion or transplantation) or due to maternal-fetal incompatibility. Objective: To characterize the donors with positive irregular antibody screening of a blood bank in Monteria, Colombia during the period 2012-2015. Methods: Cross-sectional and retrospective study, with secondary information source, based on the results of the antibody screening in donors of a blood bank in Monteria, Colombia from 2012 to 2015. The population consisted of all voluntary donors registered in the study time (35 248 donors), who were screened for antibodies. As a sample, all cases that had positive results (71 donors) were selected. The data was organized in tables and analyzed in the software SPSS 21.0, Microsoft Excel and in Epidat version 3.1. Results: 0.2 percent of the population presented a positive antibody screen with a confidence interval between 0.15 and 0.25 percent Irregular antibodies were more frequent in men and in O Rh positive donors. Thirteen types of irregular antibodies were found, with predominance of anti-M, anti-Lea, anti-D and anti-E and respective percentages of 27.78 percent, 20.83 percent, 9.72 percent and 8.33 percent. 50 percent of the donors were 30.5 years old or less, 49.3 percent had previously donated and 9.9 percent received at least one transfusion at some point in their lives. Conclusion: The frequency of donors with irregular positive antibody screening was low, the male sex had a higher percentage, it was detected mainly in blood group O and within the irregular antibodies, anti-M showed a higher frequency(AU)

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BACKGROUND: Unexpected red blood cell alloantibodies can cause hemolytic transfusion reactions. In this study, the prevalence of alloimmunization, the rate of identification of alloantibodies and the rate of blood transfusion reactions among transfused patients were identified in a clinical emergency hospital in Brazil. METHODS: Transfusions and clinical records of patients who had a positive indirect antiglobulin test between January and December 2013 were analyzed. RESULTS: Of 1169 patients who received blood transfusions, 28 had positive indirect antiglobulin tests, with one patient having two positive tests at different times, resulting in 29 positive tests during the period of this study. Alloantibodies were identified in 58.6% (17/29) of the cases. In 27.5% (8/29), identification was inconclusive and it was not possible to confirm alloimmunization. The rate of red blood cell alloimmunization was 1.71% (21/1169). Of 21 cases of alloimmunization, four (19%) were unidentified due to an unusual agglutination profile. All identified alloantibodies were clinically significant (10/17 anti-Rh, 5/17 anti-Kell and 2/17 anti-MNS). In two patients who had positive indirect antiglobulin tests, one had an unidentified alloantibody, and the other had an inconclusive test and developed a hemolytic transfusion reaction. CONCLUSION: The prevalence of clinically important red blood cell alloantibodies and hemolytic transfusion reactions among patients with unidentified alloantibodies suggests that specific laboratory techniques should be performed to identify alloantibodies in cases of pan-reactivity or autoantibodies to improve transfusion safety.

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ABSTRACT Background: Unexpected red blood cell alloantibodies can cause hemolytic transfusion reactions. In this study, the prevalence of alloimmunization, the rate of identification of alloantibodies and the rate of blood transfusion reactions among transfused patients were identified in a clinical emergency hospital in Brazil. Methods: Transfusions and clinical records of patients who had a positive indirect antiglobulin test between January and December 2013 were analyzed. Results: Of 1169 patients who received blood transfusions, 28 had positive indirect antiglobulin tests, with one patient having two positive tests at different times, resulting in 29 positive tests during the period of this study. Alloantibodies were identified in 58.6% (17/29) of the cases. In 27.5% (8/29), identification was inconclusive and it was not possible to confirm alloimmunization. The rate of red blood cell alloimmunization was 1.71% (21/1169). Of 21 cases of alloimmunization, four (19%) were unidentified due to an unusual agglutination profile. All identified alloantibodies were clinically significant (10/17 anti-Rh, 5/17 anti-Kell and 2/17 anti-MNS). In two patients who had positive indirect antiglobulin tests, one had an unidentified alloantibody, and the other had an inconclusive test and developed a hemolytic transfusion reaction. Conclusion: The prevalence of clinically important red blood cell alloantibodies and hemolytic transfusion reactions among patients with unidentified alloantibodies suggests that specific laboratory techniques should be performed to identify alloantibodies in cases of pan-reactivity or autoantibodies to improve transfusion safety.

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The shortage of dog blood donors in veterinary emergencies can lead to blood transfusions between animals whose blood type has not been identified. The antibody profile serves as a warning sign for animals that require a second blood transfusion, which is only advisable from compatible donor dogs. This article focuses on determination of anti-DEA 1 antibodies using the flow cytometry technique in dogs that have undergone a transfusion using DEA 1-positive blood, compared to results obtained from crossmatching. Blood from 18 DEA 1-positive donors ranked according to the chromatographic technique was used to transfuse thirty-three animals with unknown blood types and which demonstrated negative crossmatching to donors. On post-transfusion days 7, 14, 21 and 28, 45% and 27% of the animals tested positive for the anti-DEA 1 antibody, through crossmatching and flow cytometry, respectively. Detecting antibodies using the flow cytometric technique has high specificity and sensitivity, while crossmatching methods are highly sensitive but manifest low specificity. Following blood transfusion, animals that did not present as positive through crossmatching or flow cytometry were considered different from all other DEA 1-positive blood groups.(AU)

A escassez de cães doadores de sangue em situações de emergência na Medicina Veterinária pode levar a realização de transfusões de sangue entre animais que não tiveram seu tipo sanguíneo previamente determinado. O padrão de anticorpos serve como um sinal de alerta para animais que serão submetidos a uma segunda transfusão sanguínea, sendo essa somente recomendável a partir de cães doadores compatíveis. Este artigo aborda a pesquisa de anticorpos anti-AEC 1 pela técnica de citometria de fluxo em cães que receberam uma transfusão utilizando sangue do grupo AEC 1 positivo, comparando os resultados com aqueles obtidos a partir de reação cruzada. Foi utilizado sangue de 18 animais doadores do tipo AEC 1 positivo classificados por técnica cromatográfica para transfundir trinta e três animais com tipos sanguíneos desconhecidos, os quais mostraram reação cruzada negativa aos doadores. Nos dias 7, 14, 21 e 28 pós-transfusão, 45% e 27% dos animais mostraram-se positivos para os anticorpos anti-AEC 1, respectivamente, pela reação cruzada e através de citometria de fluxo. A pesquisa de anticorpos com o emprego da técnica de citometria de fluxo tem alta especificidade e sensibilidade, enquanto a reação cruzada, altamente sensível, tem baixa especificidade. Animais que não apresentaram positividade após a transfusão de sangue na reação cruzada e na citometria de fluxo concomitantemente foram considerados de qualquer outro grupo sanguíneo diferente do grupo sanguíneo AEC 1 positivo.(AU)

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RESUMO

The shortage of dog blood donors in veterinary emergencies can lead to blood transfusions between animals whose blood type has not been identified. The antibody profile serves as a warning sign for animals that require a second blood transfusion, which is only advisable from compatible donor dogs. This article focuses on determination of anti-DEA 1 antibodies using the flow cytometry technique in dogs that have undergone a transfusion using DEA 1-positive blood, compared to results obtained from crossmatching. Blood from 18 DEA 1-positive donors ranked according to the chromatographic technique was used to transfuse thirty-three animals with unknown blood types and which demonstrated negative crossmatching to donors. On post-transfusion days 7, 14, 21 and 28, 45% and 27% of the animals tested positive for the anti-DEA 1 antibody, through crossmatching and flow cytometry, respectively. Detecting antibodies using the flow cytometric technique has high specificity and sensitivity, while crossmatching methods are highly sensitive but manifest low specificity. Following blood transfusion, animals that did not present as positive through crossmatching or flow cytometry were considered different from all other DEA 1-positive blood groups.(AU)

A escassez de cães doadores de sangue em situações de emergência na Medicina Veterinária pode levar a realização de transfusões de sangue entre animais que não tiveram seu tipo sanguíneo previamente determinado. O padrão de anticorpos serve como um sinal de alerta para animais que serão submetidos a uma segunda transfusão sanguínea, sendo essa somente recomendável a partir de cães doadores compatíveis. Este artigo aborda a pesquisa de anticorpos anti-AEC 1 pela técnica de citometria de fluxo em cães que receberam uma transfusão utilizando sangue do grupo AEC 1 positivo, comparando os resultados com aqueles obtidos a partir de reação cruzada. Foi utilizado sangue de 18 animais doadores do tipo AEC 1 positivo classificados por técnica cromatográfica para transfundir trinta e três animais com tipos sanguíneos desconhecidos, os quais mostraram reação cruzada negativa aos doadores. Nos dias 7, 14, 21 e 28 pós-transfusão, 45% e 27% dos animais mostraram-se positivos para os anticorpos anti-AEC 1, respectivamente, pela reação cruzada e através de citometria de fluxo. A pesquisa de anticorpos com o emprego da técnica de citometria de fluxo tem alta especificidade e sensibilidade, enquanto a reação cruzada, altamente sensível, tem baixa especificidade. Animais que não apresentaram positividade após a transfusão de sangue na reação cruzada e na citometria de fluxo concomitantemente foram considerados de qualquer outro grupo sanguíneo diferente do grupo sanguíneo AEC 1 positivo.(AU)

Assuntos

RESUMO

The shortage of dog blood donors in veterinary emergencies can lead to blood transfusions between animals whose blood type has not been identified. The antibody profile serves as a warning sign for animals that require a second blood transfusion, which is only advisable from compatible donor dogs. This article focuses on determination of anti-DEA 1 antibodies using the flow cytometry technique in dogs that have undergone a transfusion using DEA 1-positive blood, compared to results obtained from crossmatching. Blood from 18 DEA 1-positive donors ranked according to the chromatographic technique was used to transfuse thirty-three animals with unknown blood types and which demonstrated negative crossmatching to donors. On post-transfusion days 7, 14, 21 and 28, 45% and 27% of the animals tested positive for the anti-DEA 1 antibody, through crossmatching and flow cytometry, respectively. Detecting antibodies using the flow cytometric technique has high specificity and sensitivity, while crossmatching methods are highly sensitive but manifest low specificity. Following blood transfusion, animals that did not present as positive through crossmatching or flow cytometry were considered different from all other DEA 1-positive blood groups.

A escassez de cães doadores de sangue em situações de emergência na Medicina Veterinária pode levar a realização de transfusões de sangue entre animais que não tiveram seu tipo sanguíneo previamente determinado. O padrão de anticorpos serve como um sinal de alerta para animais que serão submetidos a uma segunda transfusão sanguínea, sendo essa somente recomendável a partir de cães doadores compatíveis. Este artigo aborda a pesquisa de anticorpos anti-AEC 1 pela técnica de citometria de fluxo em cães que receberam uma transfusão utilizando sangue do grupo AEC 1 positivo, comparando os resultados com aqueles obtidos a partir de reação cruzada. Foi utilizado sangue de 18 animais doadores do tipo AEC 1 positivo classificados por técnica cromatográfica para transfundir trinta e três animais com tipos sanguíneos desconhecidos, os quais mostraram reação cruzada negativa aos doadores. Nos dias 7, 14, 21 e 28 pós-transfusão, 45% e 27% dos animais mostraram-se positivos para os anticorpos anti-AEC 1, respectivamente, pela reação cruzada e através de citometria de fluxo. A pesquisa de anticorpos com o emprego da técnica de citometria de fluxo tem alta especificidade e sensibilidade, enquanto a reação cruzada, altamente sensível, tem baixa especificidade. Animais que não apresentaram positividade após a transfusão de sangue na reação cruzada e na citometria de fluxo concomitantemente foram

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BACKGROUND: Based upon serology, >10 canine blood group systems have been reported. OBJECTIVE: We surveyed dogs for dog erythrocyte antigen (DEA) 1 and 2 new blood types (Kai 1 and Kai 2), and some samples also were screened for Dal and DEA 3, 4, and 7. METHODS: Blood samples provided by owners, breeders, animal blood banks, and clinical laboratories were typed for DEA 1 by an immunochromatographic strip technique with a monoclonal antibody and analysis of band intensity. Both new antigens, the Dal and other DEAs (except DEA 7 by tube method), were assessed by a gel column method with either monoclonal or polyclonal antibodies. The same gel column method was applied for alloantibody detection. RESULTS: Of 503 dogs typed, 59.6% were DEA 1+ with 4% weakly, 10% moderately, and 45.6% strongly DEA 1+. Regarding Kai 1 and Kai 2, 94% were Kai 1+/Kai 2-, 5% were Kai 1-/Kai 2- and 1% were Kai 1-/Kai 2+, but none were Kai 1+/Kai 2+. There was no relationship between Kai 1/Kai 2 and other blood types tested. Plasma from DEA 1-, Kai 1-, Kai 2- dogs, or some combination of these contained no detectable alloantibodies against DEA 1 and Kai 1 or Kai, respectively. CONCLUSIONS AND CLINICAL IMPORTANCE: The new blood types, called Kai 1 and Kai 2, are unrelated to DEA 1, 3, 4, and 7 and Dal. Kai 1+/Kai 2- dogs were most commonly found in North America. The clinical relevance of Kai 1 and Kai 2 in canine transfusion medicine still needs to be elucidated.

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