Paciente con anemia y síndrome de Rendu-osler-weber
2021
espanolVaron de 66 anos con Telangiectasia Hemorragica Hereditaria tipo I que consulta por astenia intensa detectandose en analitica Hb 4 mg/dl; VCM 100,5%. El paciente no refiere sintomas de sangrado. En gastroscopia se visualizan multiples malformaciones vasculares de tamano variable y sin sangrado activo en esofago, estomago, siendo mas numerosas en duodeno. En colonoscopia se distinguen varias angiodisplasias sin estigmas de sangrado reciente en ciego. El paciente recibe tratamiento de forma periodica con hierro intravenoso y transfusiones de concentrados de hematies. La Telangiectasia Hemorragica Hereditaria o sindrome de Rendu-Osler-Weber es una enfermedad hereditaria rara, de transmision autosomica dominante, caracterizada por la aparicion de malformaciones arterio-venosas o telangiectasias en cualquier localizacion del organismo. El sintoma mas frecuente es la epistaxis asociada a anemia, seguida del sangrado gastrointestinal en el 13-30% de los pacientes. El tratamiento es de soporte, siendo importante tratar la anemia con hierro y transfusiones sanguineas. Durante la endoscopia, cabe destacar la importancia de identificar el punto de sangrado activo y realizar sobre el mismo tratamiento de coagulacion con plasma argon. English66-year-old man with Hereditary Hemorrhagic Telangiectasia type I who consulted for severe asthenia, detecting Hb 4 mg/dl; VCM 100.5% in blood tests. The patient does not report symptoms of bleeding. Gastroscopy shows multiple vascular malformations of variable size and without active bleeding in the esophagus, stomach, being more numerous in the duodenum. In colonoscopy, several angiodysplasias without stigmata of recent bleeding in the cecum are distinguished. The patient is regularly treated with intravenous iron and packed red blood cell transfusions. Hereditary Hemorrhagic Telangiectasia or Rendu-Osler-Weber syndrome is a rare hereditary disease, of autosomal dominant transmission, characterized by the appearance of arterio-venous malformations or telangiectasias in any location of the body. The most frequent symptom is epistaxis associated with anemia, followed by gastrointestinal bleeding in 13-30% of patients. Treatment is supportive, being important to treat anemia with iron and blood transfusions. During endoscopy, it is important to highlight the importance of identifying the active bleeding point and to perform argon plasma coagulation treatment on the same.
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