Frequency of paroxysmal nocturnal hemoglobinuria in patients attended in Belém, Pará, Brazil
Rev. bras. hematol. hemoter
;
33(1): 35-37, Feb. 2011. []
Artigo
em Inglês
| LILACS
| ID: lil-582745
ABSTRACT
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).
Texto completo:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Imunofenotipagem
/
Antígenos CD59
/
Antígenos CD55
/
Citometria de Fluxo
/
Hemoglobinúria Paroxística
Tipo de estudo:
Estudo prognóstico
Limite:
Adolescente
/
Adulto
/
Idoso
/
Criança
/
Criança, pré-escolar
/
Feminino
/
Humanos
/
Masculino
País/Região como assunto:
América do Sul
/
Brasil
Idioma:
Inglês
Revista:
Rev. bras. hematol. hemoter
Assunto da revista:
Hematologia
Ano de publicação:
2011
Tipo de documento:
Artigo
País de afiliação:
Brasil
Instituição/País de afiliação:
Fundação Centro de Hematologia e Hemoterapia do Pará/BR
/
Universidade Federal do Pará/BR
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