Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Disease) Management of Epistaxis in Nine Patients Using Systemic Hormone Therapy

Hormonal and Antihormonal Therapy for Epistaxis in Hereditary Hemorrhagic Telangiectasia

“…While probably effective, 13–16 high‐dose estrogen therapy should not be considered in essentially all patients because of the potentially serious side effects. The controlled study by Van Cutsem et al 19 in HHT‐related gastrointestinal tract bleeding, together with the uncontrolled trial of Flessa and Glueck 17 in HHT‐related epistaxis, provides support for the use of estrogen‐progesterone therapy in symptomatic HHT at oral contraceptive doses. The relative safety of contemporary oral contraceptive medications, particularly in nonsmoking female patients under 35 years of age, would make this logical as an initial therapeutic recommendation for symptomatic women of reproductive age.…”

“…Instead of single‐agent estrogen for epistaxis in HHT, Flessa and Glueck, 17 in an uncontrolled trial, treated eight women and one man with the combination oral contraceptive Enovid containing both estrogen and progesterone. Women were treated with cyclical therapy, and the man received the drugs daily without interruption.…”

“…Side effects or complications related to or possibly associated with hormone therapy were seen in seven of the nine patients (Table I). 17 …”

A Case Report of a Patient with Hereditary Hemorrhagic Telangiectasia Treated Successively with Thalidomide and Bevacizumab

“…In addition, these patients must receive erythrocyte concentrates and iron substitution. Gastrointestinal bleeding in HHT patients could be reduced by administration of an estrogen-progesterone combination [31]. This therapy has the advantage that the gastrointestinal sections are treated, which cannot be examined endoscopically.…”

Antiestrogen therapy for hereditary hemorrhagic telangiectasia: A double‐blind placebo‐controlled clinical trial

“…At present, estrogen is the most popular pharmacologic treatment for HHT‐associated telangiectasis, with favorable results reported in several studies 13–17, 19. The rationale for its use is based on estrogen's ability to provoke squamous epithelial metaplasia, which protects superficial telangiectacic vessels from breaking down and bleeding 23.…”