Diagnóstico por citometria de fluxo da hemoglobinúria paroxística noturna
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Data
2023-06-22
Tipo de documento
Artigo Científico
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Título de Volume
Área do conhecimento
Ciências da Saúde
Modalidade de acesso
Acesso aberto
Editora
Autores
Ferreira, Gabriela
Lucena, Vitória
Orientador
Carobin, Natália
Coorientador
Resumo
A hemoglobinúria paroxística noturna (HPN) é caracterizada por uma doença hemolítica crônica rara adquirida. A HPN pode surgir juntamente com outras doenças hematológicas e achados laboratoriais, como trombocitopenia , neutropenia e síndromes de falência medular. A HPN é uma doença adquirida em decorrência de uma mutação somática no gene PIG-A, isso ocorre nas células tronco, ocasionando na ausência de CD59 na superfície dos eritrócitos tornando os mesmos vulneráveis a lise mediada pelo sistema complemento. Dessa forma, podemos observar na atualidade as deficiências dos testes de triagem para HPN (testes Ham, pesquisas de CD59 e CD55 em coluna em gel), sendo o método mais utilizado na atualidade a citometria de fluxo (CMF), este que é um teste capaz de quantificar e detectar o clone da HPN com mais rapidez e eficácia, assim ajudando em um melhor prognóstico. Os diagnósticos e resultados realizados através do método de citometria de fluxo comprovam o motivo da limitação dos outros testes de triagem, demonstrando o lado vantajoso da citometria de fluxo em identificar variações e intensidades dos clones, garantindo um diagnóstico mais certo e preciso.
Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by a rare acquired chronic hemolytic disease. This disease arises along with other hematological diseases. It often presents with infections, these infections are often recurrent, thrombocytopenia and neutropenia, especially with bone marrow failure syndromes. This disease arises in association with other types of diseases, such as myelodysplastic syndrome and aplastic anemia. PNH is a disease acquired as a result of a somatic mutation in the PIG-A gene, this occurs in stem cells, causing a failure of erythrocytes, leukocytes and platelets to protect themselves against lysis that are measured by the complement system. Thus, we can currently observe the deficiencies of screening tests for PNH (Ham tests, CD59 and CD55 research in gel column), ceasing to use these tests, and starting to use the test by the flow cytometry method (CMF) , which is a test capable of quantifying and detecting PNH clones more quickly and efficiently, thus helping to improve the prognosis. The diagnoses and results performed using the flow cytometry method prove the reason for the low use and limitation of other screening tests, and demonstrate the advantage of identifying variations and intensities of clones, ensuring a more accurate and accurate diagnosis.
Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by a rare acquired chronic hemolytic disease. This disease arises along with other hematological diseases. It often presents with infections, these infections are often recurrent, thrombocytopenia and neutropenia, especially with bone marrow failure syndromes. This disease arises in association with other types of diseases, such as myelodysplastic syndrome and aplastic anemia. PNH is a disease acquired as a result of a somatic mutation in the PIG-A gene, this occurs in stem cells, causing a failure of erythrocytes, leukocytes and platelets to protect themselves against lysis that are measured by the complement system. Thus, we can currently observe the deficiencies of screening tests for PNH (Ham tests, CD59 and CD55 research in gel column), ceasing to use these tests, and starting to use the test by the flow cytometry method (CMF) , which is a test capable of quantifying and detecting PNH clones more quickly and efficiently, thus helping to improve the prognosis. The diagnoses and results performed using the flow cytometry method prove the reason for the low use and limitation of other screening tests, and demonstrate the advantage of identifying variations and intensities of clones, ensuring a more accurate and accurate diagnosis.
Palavras-chave
Glicosil-fosfatidil inositol, Fosfatidilinositol glicano classe A, Hemoglobinúria Paroxística Noturna, Citometria de Fluxo