Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil
BACKGROUND: Paroxysmal nocturnal hemoglobinuria is a hematological disease with complex physiopathology. It is genetically characterized by a somatic mutation in the PIG-A gene (phosphatidylinositol glycan anchor biosynthesis, class A), in which the best known antigens are DAF (decay accelerating fa...
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| Format: | Article |
| Language: | English |
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Elsevier
2011-02-01
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| Series: | Revista Brasileira de Hematologia e Hemoterapia |
| Subjects: | |
| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842011000100012 |
| _version_ | 1811309948341059584 |
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| author | Lacy Cardoso de Brito Junior Maria do Socorro de Oliveira Cardoso Euzamar Gaby Rocha Herika Anijar Mariana Cunha João Carlos Pina Saraiva |
| author_facet | Lacy Cardoso de Brito Junior Maria do Socorro de Oliveira Cardoso Euzamar Gaby Rocha Herika Anijar Mariana Cunha João Carlos Pina Saraiva |
| author_sort | Lacy Cardoso de Brito Junior |
| collection | DOAJ |
| description | BACKGROUND: Paroxysmal nocturnal hemoglobinuria is a hematological disease with complex physiopathology. It is genetically characterized by a somatic mutation in the PIG-A gene (phosphatidylinositol glycan anchor biosynthesis, class A), in which the best known antigens are DAF (decay accelerating factor or CD55) and MIRL (membrane inhibitor of reactive lysis or CD59). OBJECTIVE: To determine the frequency of paroxysmal nocturnal hemoglobinuria in patients attended at the HEMOPA foundation from November 2008 to July 2009. METHOD: Thirty patients, with ages ranging from two to 79 years old and suspected of having paroxysmal nocturnal hemoglobinuria were examined. All patients were immunophenotyped by flow cytometry for the CD5, CD59, CD16 and CD45 antigens. RESULTS: Paroxysmal nocturnal hemoglobinuria was identified in nine of the thirty patients investigated. Another 3 cases had inconclusive results with CD59-negative labeling only for neutrophils. The highest frequency of paroxysmal nocturnal hemoglobinuria patients (7/9) and inconclusive cases (2/3) were between 19 years old and 48 years old, with a median of 28 years. CONCLUSION: These results show the importance of flow cytometry to identify cases in which patients are deficient in only one antigen (CD59). |
| first_indexed | 2024-04-13T09:49:50Z |
| format | Article |
| id | doaj.art-b45a230525d84135ba55200713d15667 |
| institution | Directory Open Access Journal |
| issn | 1516-8484 1806-0870 |
| language | English |
| last_indexed | 2024-04-13T09:49:50Z |
| publishDate | 2011-02-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Revista Brasileira de Hematologia e Hemoterapia |
| spelling | doaj.art-b45a230525d84135ba55200713d156672022-12-22T02:51:37ZengElsevierRevista Brasileira de Hematologia e Hemoterapia1516-84841806-08702011-02-01331353710.5581/1516-8484.20110012Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, BrazilLacy Cardoso de Brito JuniorMaria do Socorro de Oliveira CardosoEuzamar Gaby RochaHerika AnijarMariana CunhaJoão Carlos Pina SaraivaBACKGROUND: Paroxysmal nocturnal hemoglobinuria is a hematological disease with complex physiopathology. It is genetically characterized by a somatic mutation in the PIG-A gene (phosphatidylinositol glycan anchor biosynthesis, class A), in which the best known antigens are DAF (decay accelerating factor or CD55) and MIRL (membrane inhibitor of reactive lysis or CD59). OBJECTIVE: To determine the frequency of paroxysmal nocturnal hemoglobinuria in patients attended at the HEMOPA foundation from November 2008 to July 2009. METHOD: Thirty patients, with ages ranging from two to 79 years old and suspected of having paroxysmal nocturnal hemoglobinuria were examined. All patients were immunophenotyped by flow cytometry for the CD5, CD59, CD16 and CD45 antigens. RESULTS: Paroxysmal nocturnal hemoglobinuria was identified in nine of the thirty patients investigated. Another 3 cases had inconclusive results with CD59-negative labeling only for neutrophils. The highest frequency of paroxysmal nocturnal hemoglobinuria patients (7/9) and inconclusive cases (2/3) were between 19 years old and 48 years old, with a median of 28 years. CONCLUSION: These results show the importance of flow cytometry to identify cases in which patients are deficient in only one antigen (CD59).http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842011000100012ImmunophenotypingFlow cytometryParoxysmal nocturnal hemoglobinuriaDiagnosisAntigen, CD55Antigen, CD59 |
| spellingShingle | Lacy Cardoso de Brito Junior Maria do Socorro de Oliveira Cardoso Euzamar Gaby Rocha Herika Anijar Mariana Cunha João Carlos Pina Saraiva Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil Revista Brasileira de Hematologia e Hemoterapia Immunophenotyping Flow cytometry Paroxysmal nocturnal hemoglobinuria Diagnosis Antigen, CD55 Antigen, CD59 |
| title | Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil |
| title_full | Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil |
| title_fullStr | Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil |
| title_full_unstemmed | Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil |
| title_short | Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil |
| title_sort | frequency of paroxysmal nocturnal hemoglobinuria in patients attended in belem para brazil |
| topic | Immunophenotyping Flow cytometry Paroxysmal nocturnal hemoglobinuria Diagnosis Antigen, CD55 Antigen, CD59 |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842011000100012 |
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