Multidisciplinary management of anemia behind epistaxis in HHT

HHT affects one in 5000 people and occurs in all the ethnic groups and areas. It is also known as the Rendu-Osler-Weber disease and it is an inherited autosomal dominant genetic disorder, characterized by vascular abnormalities. Epistaxis, specifically recurrent and spontaneous nosebleeds, has been assessed as one of the most common, if not the most common clinical manifestation in HHT patients. The burden related to this manifestation has both psychological and physical consequences, especially since the treatment options follow a ladder that might bring to surgery and more invasive therapies. The EQ-VAS questionnaire allows us to adequately assess and classify HHT patients based on the intensity and type of epistaxis-related symptoms. This same questionnaire, which is submitted to patients during each evaluation for the benefit of anamnestic supplementation, includes both a question about the presence or absence of anemia and one about whether a red cell transfusion has been performed in the past months or since the last outpatient visit. As a matter of fact, chronic nosebleed, although mild to moderate, can lead to anemia within months or years and, in general, to a poor quality of life. Patients who have to undergo iron supplementation treatments often face the almost inevitable side effects that this therapy entails (diarrhea, constipation, nausea, persistent metallic taste, abdominal pain, etc.). Although numerous treatment options are available for patients with epistaxis phenotype, from topical to surgical, we believe, based on the successes achieved in the follow up of HHT patients at our center, that a multidisciplinary collaboration is essential to identify the patients who can benefit most from each treatment.