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Acta Haematol ; 123(1): 37-42, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-19940469

RESUMO

INTRODUCTION: Patients with thalassemia major often present with a hypercoagulable state, the pathogenesis of which is still not understood. MATERIALS AND METHODS: This study evaluates the risk factors for hypercoagulability in 50 beta-thalassemia major patients and 50 healthy controls. Fasting total homocysteine, protein C (PC), protein S (PS), antithrombin (AT), activated protein C resistance (APCR) and lupus anticoagulant (LA) were assessed. MTHFR C677T mutation was determined. RESULTS: Significant reductions in PC, PS and AT were noted in patients. Only 4% of the patients had hyperhomocysteinemia. Thirty-two percent of the patients were heterozygous and 4% were homozygous for MTHFR C677T mutation. CONCLUSION: The natural coagulation inhibitors PC, PS and AT were significantly reduced in patients with beta-thalassemia major and were thus important risk factors for the hypercoagulable state, but hyperhomocysteinemia and MTHFR mutation do not seem to be significant risk factors for thromboembolic events.


Assuntos
Transtornos da Coagulação Sanguínea/sangue , Transtornos da Coagulação Sanguínea/etiologia , Metilenotetra-Hidrofolato Redutase (NADPH2)/genética , Mutação Puntual , Talassemia beta/sangue , Talassemia beta/genética , Resistência à Proteína C Ativada/sangue , Adolescente , Adulto , Antitrombinas/metabolismo , Sequência de Bases , Transtornos da Coagulação Sanguínea/genética , Estudos de Casos e Controles , Primers do DNA/genética , Feminino , Heterozigoto , Homocisteína/sangue , Homozigoto , Humanos , Kuweit , Inibidor de Coagulação do Lúpus/sangue , Masculino , Proteína C/metabolismo , Proteína S/metabolismo , Fatores de Risco , Tromboembolia/sangue , Tromboembolia/etiologia , Tromboembolia/genética , Adulto Jovem , Talassemia beta/complicações
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