Hemofilia A adquirida: reporte de un caso
Autores
Esteban Castro, Joaquín Marchena, Juan José Rivas-Mejías, Jusara Ortiz, Lilliana Calvo, Luisa Vindas, María Fernanda Sánchez
Resumen
La hemofilia A adquirida (HAA) es un trastorno hemorrágico raro, sin embargo, es muy severo. Se caracteriza por la presencia de autoanticuerpos contra el factor VIII de la coagulación. A pesar de que el 50% de los autoanticuerpos son de origen idiopático, existen factores de riesgo reconocidos para la HAA, como la presencia de enfermedades autoinmunes. Se presenta el caso clínico de una paciente femenina de 55 años, con historial de 10 años de artritis reumatoide, que consulta por equimosis grandes y dolorosas en miembro superior izquierdo, sin trauma previo. Al realizar exámenes de laboratorio, se determina un tiempo de tromboplastina parcial activado prolongado, por lo que se realizan otros estudios; finalmente, se encuentra disminución del factor VIII y método de Kasper positivo, lo que, confirma la sospecha clínica de HAA. La paciente respondió al tratamiento con factor VII recombinante, prednisona y ciclofosmamida, y se mantiene con profilaxis. El caso concuerda con lo reportado en otros estudios dada la enfermedad auotinmune de fondo, la hemostasia previa normal y la inexistencia de historial de sangrado en la paciente. Las pruebas de laboratorio concuerdan con el diagnóstico, descartan los principales diagnósticos diferenciales y permiten el manejo clínico oportuno de la enfermedad.
Palabras clave
Hemofilia A adquirida, deficiencia de factor VIII adquirida, inhibidores contra factores de coagulación.Abstract
Acquired hemophilia A (AHA) is a rare but severe hemorrhagic disorder characterized by autoantibodies against coagulation factor VIII. Although 50% of autoantibodies have an idiopathic origin, there are well recognized risk factors for AHA, such as autoimmune diseases. We present the clinical case of a 55-year-old female patient, with a 10-year history of rheumatoid arthritis, who consulted for large, painful bruises on the left upper extremity without prior trauma. Within the laboratory tests, a prolonged activated partial thromboplastin was found; then, other studies were carried out; finally, a decrease in factor VIII, and a positive Kasper method confirmed the clinical suspicion of AHA. The treatment with recombinant factor VII, prednisone and cyclophosphamide was successful, and was kept with prophylaxis. This case matches other reports in literature given the underlying autoimmune disease, the normal previous haemostasis, and the lack of history of bleeding. The laboratory tests correlate with diagnosis, discard the main differential diagnoses, and allow a proper clinical management of the disease.
Key words
Acquired hemophilia A, acquired Factor VIII deficiency, blood coagulation factor inhibitors.Texto completo
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