RESUMO
BACKGROUND AND OBJECTIVES: Mucocutaneous bleeding (MCB) is the main expression of inherited disorders of primary hemostasis. However, the relative prevalence of these disorders, their clinical differential diagnosis, and the proportion of patients with MCB of unknown cause (BUC) after an initial comprehensive laboratory testing are unknown. DESIGN AND METHODS: We studied prospectively 280 consecutive patients with MCB and 299 matched controls, using strict inclusion and exclusion criteria. A single physician recorded the clinical data in a bleeding score and estimated the severity of bleeding in clinical categories. Laboratory criteria for the diagnosis of von Willebrand's disease (VWD) and platelet function defects were established from reference values derived from controls. RESULTS: Fifty patients (17.9%) had VWD (type 1VWD=45, type 2=5). Platelet function defects and mild clotting factor deficiencies were found in 65 (23.2%) and 11 (3.9%) patients, respectively. Thirteen (11.5%) patients had combined defects. The remaining 167(59.6%) patients had BUC, with prolonged bleeding time in 18.6% as their only abnormality. All these disorders, including BUC, were clinically undistinguishable. Moreover, no relationship was found between the severity of bleeding and VWF/platelet function variables. INTERPRETATION AND CONCLUSIONS: The diagnostic efficacy of a first laboratory testing in patients with hereditary MCB is 40.4%. Most patients have a disease(s) of high prevalence but unknown pathogenesis. Concurrent bleeding disorders in the same patient are frequent. Our results support the proposal that low plasma VWF levels, but also platelet function defects, should be considered risk factors rather than unequivocal causes of hemorrhages.
Assuntos
Hemorragia/etiologia , Transtornos Hemorrágicos/diagnóstico , Mucosa , Dermatopatias/etiologia , Adolescente , Adulto , Tempo de Sangramento , Transtornos Plaquetários/sangue , Transtornos Plaquetários/complicações , Transtornos Plaquetários/diagnóstico , Transtornos Plaquetários/epidemiologia , Plaquetas/efeitos dos fármacos , Plaquetas/metabolismo , Administração de Caso , Estudos de Casos e Controles , Criança , Pré-Escolar , Transtornos de Proteínas de Coagulação/sangue , Transtornos de Proteínas de Coagulação/complicações , Transtornos de Proteínas de Coagulação/diagnóstico , Transtornos de Proteínas de Coagulação/epidemiologia , Epinefrina/farmacologia , Feminino , Hemoglobinas/análise , Hemorragia/sangue , Transtornos Hemorrágicos/sangue , Transtornos Hemorrágicos/complicações , Transtornos Hemorrágicos/epidemiologia , Transtornos Hemorrágicos/genética , Humanos , Masculino , Anamnese , Pessoa de Meia-Idade , Fenótipo , Testes de Função Plaquetária/instrumentação , Testes de Função Plaquetária/métodos , Valor Preditivo dos Testes , Prevalência , Estudos Prospectivos , Serotonina/metabolismo , Índice de Gravidade de Doença , Transdução de Sinais , Espanha/epidemiologia , Inquéritos e Questionários , Doenças de von Willebrand/sangue , Doenças de von Willebrand/classificação , Doenças de von Willebrand/complicações , Doenças de von Willebrand/diagnóstico , Doenças de von Willebrand/epidemiologiaRESUMO
We compared the effects of two third-generation progestogens, desogestrel (DSG) and gestodene (GSD), on coagulation and fibrinolysis in Brazilian users of oral contraceptives (OCs). Forty-six women were evaluated before treatment and after six cycles of treatment. The coagulation, anticoagulant, and fibrinolytic systems were investigated. During the use of the DSG-containing OC, the activity of factors VII, VIII, IX, X, and XII increased significantly whereas the GSD-containing OC caused no changes in coagulation parameters. Concerning the anticoagulant pathways, the DSG-containing OC increased protein C levels and decreased total protein S levels, and the GSD-containing OC only decreased total protein S. Both OCs increased plasminogen activity, although the DSG-containing OC increased fibrin degradation products levels and decreased the tissue plasminogen activator antigen. In conclusion, we have found that in Brazilian women the effects of DSG and GSD on hemostatic parameters are different and, therefore, third-generation progestogens may not contribute equally to the thrombotic risk.