About this trial
Clinical Study Abstract (Ethics Application) Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant vascular disorder characterized by recurrent intractable epistaxis and multi-organ arteriovenous malformations. Moderate-to-severe patients often develop refractory anemia with severely impaired quality of life. Current stepwise therapeutic strategies have substantial limitations, including frequent adverse reactions of thalidomide, high recurrence rates after electrocoagulation, and severe surgical trauma, resulting in a lack of safe and individualized treatment options. Our preliminary clinical practice has verified the promising efficacy and long-term benefits of bevacizumab in severe HHT-related epistaxis. However, obvious interindividual variability and occasional adverse events exist, and the underlying mechanism remains unclear. This study aims to systematically evaluate the efficacy and safety of bevacizumab for severe HHT-associated epistaxis, explore the influences of baseline clinical and genetic factors on prognosis, and investigate the mechanism of treatment heterogeneity, so as to optimize individualized therapeutic strategies and provide clinical evidence for precise management of HHT in China.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
Definite HHT diagnosis is established by either clinical assessment according to the Curaçao criteria or genetic confirmation.
Disqualifiers
Minors, patients with mild epistaxis that can be well controlled by other treatments, and patients with any contraindication to bevacizumab treatment.
Trial design
Parallel
Treatments tested in this trial
Bevacizumab
DrugBevacizumab is administered intravenously at 5 mg/kg body weight, 1 to 2 times annually, starting one month after nasal electrocoagulation.
nasal electrocoagulation.
Procedure/Surgerynasal electrocoagulation.
Thalidomide (50mg)
DrugOral thalidomide is initiated at 50 mg twice daily (100 mg per day) one month after nasal electrocoagulation.
Treatment groups
Trial outcomes
Primary outcomes
ESS
Epistaxis Severity Score
Hb
Hemoglobin (Hb)
Sponsors and contacts
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