RESUMEN
Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder caused by von Willebrand factor (vWF) dysfunction of various types and severities. Clinical manifestations and differences in the intensity of bleeding are then given by the diversity of vWF disorders. AvWS usually occurs in adult patients with a negative personal or family history of bleeding symptoms and is associated with underlying disease. In recent years, vWF dysfunction has been most commonly attributed to cardiovascular, autoimmune, lymphoproliferative or myeloproliferative comorbidities. The true prevalence of AvWS is unknown, since many cases may be clinically or laboratory silent and remain undiagnosed. Thus, a higher incidence can be expected in the above-mentioned specific patient population. We present a case report of a 90year-old patient with the very first episode of bleeding manifestation and newly diagnosed vWF dysfunction. By describing the case, we will try to present the diagnostic and treatment options of AvWS, including their pitfalls, and draw attention to situations in which to think about AvWS.
Asunto(s)
Enfermedades de von Willebrand , Factor de von Willebrand , Adulto , Hemorragia/etiología , Humanos , Enfermedades de von Willebrand/complicaciones , Enfermedades de von Willebrand/diagnóstico , Enfermedades de von Willebrand/tratamiento farmacológico , Factor de von Willebrand/uso terapéuticoRESUMEN
Idarucizumab is designed to reverse the anticoagulant effect of dabigatran. This case report describes the administration of three independent doses of idarucizumab to a 76-year-old man suffering from atrial flutter being treated with dabigatran to prevent ischaemic stroke. The last dose of dabigatran was administered in the morning of the same day the patient was transferred to hospital because of the need for urgent pericardium puncture. Baseline dTT (dilute thrombin time) reached 700 ng/mL as glomerular filtration (GF) dropped to 0.19 mL/s. The first dose of idarucizumab was administered prior to puncture and the clinical state was stabilized for the next 24 hours although dTT climbed to 400 ng/mL. The need for cannulation before dialysis required a second dose of antidote and a third dose was given prior to removal of the pericardium drain. Monitoring of dabigatran recovery showed that the reverse effect of idarucizumab lasted 12 hours, which is sufficiently long and reliable for urgent situations. Idarucizumab administration is not limited by age and/or renal impairment. We recommend repeated dTT examinations when the dabigatran baseline level exceeds 200 ng/mL and the antidote has been administered.
Asunto(s)
Anticuerpos Monoclonales Humanizados/administración & dosificación , Aleteo Atrial/tratamiento farmacológico , Infarto Cerebral/prevención & control , Dabigatrán/efectos adversos , Sobredosis de Droga/tratamiento farmacológico , Anciano , Infarto Cerebral/inducido químicamente , Dabigatrán/administración & dosificación , Esquema de Medicación , Sobredosis de Droga/sangre , Servicios Médicos de Urgencia , Tasa de Filtración Glomerular/efectos de los fármacos , Humanos , Masculino , Pericardiectomía , Diálisis Renal , Retratamiento , Tiempo de TrombinaRESUMEN
Dabigatran (Pradaxa, Boehringer Ingelheim) is the first direct oral thrombin (FIIa) inhibitor. It is indicated for thromboprophylaxis in patients undergoing elective total replacement hip or knee joint surgery or for the primary or secondary prophylaxis of elderly patients with non-valvular atrial fibrillation or for deep vein thrombosis and pulmonary embolism treatment and prophylaxis. Idarucizumab (Praxbind, Boehringer Ingelheim) is a specific monoclonal antibody fragment indicated in patients treated with dabigatran. It is recommended when reversal of anticoagulant effect of dabigatran is needed especially in cases of life-threatening bleeding, for emergency surgery or urgent procedures purposes. In this article, there is a summary of our clinical and laboratory experience with reversal effect of idarucizumab in our five patients.