Bevacizumab in Patients With Hereditary Hemorrhagic Telangiectasia and Severe Hepatic Vascular Malformations and High Cardiac Output

Abstract

EREDITARY HEMORRHAGIC telangiectasia (HHT) (Online Mendelian Inheritance in Man [OMIM] #187300) is a dominantly inherited genetic vascular disorder characterized by recurrent epistaxis; cutaneous telangiectasia; and visceral arteriovenous malformations (AVMs) that affect many organs, including the lungs, gastrointestinal tract, liver, and brain. Diagnosis is based on the Curaçao criteria and is considered definite if at least 3 of 4 criteria are fulfilled. 1 The criteria are spontaneous and recurrent epistaxis, Author Affiliations are listed at the end of this article.

Blessing for the Bleeder: Bevacizumab in Hereditary Hemorrhagic Telangiectasia

“…2,3 Bevacizumab, a VEGF inhibitor, has been shown to reduce epistaxis, telangiectasias, and the need for iron infusions due to stabilized hemoglobin (Hgb). [3][4][5][6][7][8][9][10] Additionally, there has been marked improvement in symptoms and transfusion requirements. Other reports demonstrated improvement in symptoms and transfusion requirements without an appreciable change in AVMs.…”

“…4,6 DupuisGirod et al showed that bevacizumab administration is associated with improvement in high output cardiac failure, secondary to its effect on liver capillaries and arteriovenous shunting. 5,7 In the previously reported cases, the dose of bevacizumab varied from 5 mg per kilogram (mg/kg) body weight 1,4-10 to 10 mg/kg body weight 3 administered every 2 weeks (table 1). The elimination half-life of bevacizumab has been determined to be 18 to 20 days; however, in the study done by Dupuis-Girod et al 5 it was found to be 21.5 days.…”

“…5,7 In the previously reported cases, the dose of bevacizumab varied from 5 mg per kilogram (mg/kg) body weight 1,4-10 to 10 mg/kg body weight 3 administered every 2 weeks (table 1). The elimination half-life of bevacizumab has been determined to be 18 to 20 days; however, in the study done by Dupuis-Girod et al 5 it was found to be 21.5 days. The optimal dose and schedule of administration of bevacizumab in the treatment of HHT is unknown.…”

Hereditary hemorrhagic teleangiectasia: the topical problems of diagnostics and surgical treatment

“…Бе вацизумаб является одним из таких препаратов. Он ингибирует биологическую активность фактора роста эндотелия сосудов (VEGF) и применяется в настоя щее время как химиотерапевтический препарат [79].…”

Successful therapy with bevacizumab in a case of hereditary hemorrhagic telangiectasia

“…Taking into account some literature reports, describing positive effects of bevacizumab [2][3][4] -following the patient's consent and having got a permission of the Bioethical Commission of the District Chamber of Physicians for the use of an "off-label" drug, totally 6 i.v. injections of 200 mg doses of bevacizumab were administered during the period from February to May 2014 in the 3-week intervals.…”