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BABH Study: Efficacy and Safety of Bevacizumab on Severe Bleedings Associated With Hemorrhagic Hereditary Telangiectasia (HHT).

Phase 3
Completed
Conditions
Rendu Osler Disease
Telangiectasia, Hereditary Hemorrhagic
Interventions
Registration Number
NCT03227263
Lead Sponsor
Hospices Civils de Lyon
Brief Summary

The recognized manifestations of HHT are all due to abnormalities of vascular structure. Epistaxis and digestive arteriovenous malformations may be responsible for severe hemorrhages in 5% of HHT patients, requiring repeated blood transfusions and are associated with high morbidity. There is currently no standard and efficient management of this severe symptom. It is also well known that HHT-associated hemorrhages have the greatest negative impact on quality of life among HHT patients, and is responsible for anemia, blood transfusions, hospitalizations, depressive syndrome and a high psycho-social impact.

Since 2006, it has been suggested by animal models and then by clinical reports that anti-VEGF therapy may be useful to treat HHT. 4 case reports have been published on efficacy of intravenous bevacizumab, a humanized monoclonal antibody in HHT on severe hemorrhages.

Intravenous bevacizumab has been used in a previous clinical trial to measure efficacy and tolerance of this drug in HHT patients with severe liver involvement. Furthermore, a reduction was observed in the duration of the nosebleeds after treatment and was encouraging to treat bleeding. We completed this study by a pharmacokinetic-pharmacodynamic (PK-PD) model in order to assess the individual concentration-effect relationship of bevacizumab.

However, no randomized prospective study has been performed and published to evaluate the efficacy in this indication. A total of 24 patients will be randomized versus placebo in a multicenter phase III trial. The Avastin or placebo will be infused at 5mg/kg every 14 days with a total of 6 cures with a 3 months following period.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
24
Inclusion Criteria
  • Age ≥ 18 years.
  • Patients who have given their free informed and signed consent.
  • Patients affiliated to a social security scheme or similar.
  • Patients monitored for clinically confirmed HHT (presence of at least three Curaçao criteria) and / or with molecular biology confirmation.
  • Blood transfusions with the requirement for at least 4 units of blood in the 3-month period before study enrollment, related to epistaxis or digestive bleeding.
Read More
Exclusion Criteria
  • Women who are pregnant or nursing (lactating), women of child-bearing potential without reliable contraception during the treatment and for at least 6 months after the last dose.
  • Patients who are protected adults under the terms of the law (French Public Health Code).
  • Refusal to consent.
  • Patients for whom the diagnosis of HHT has not been confirmed clinically and / or by molecular biology study.
  • Active infection and/or fever>38°C
  • Participation in another clinical trial within 28 days prior to inclusion.
  • Hypersensitivity to the active substance or to any of the excipients.
  • Known hypersensitivity to products of Chinese hamster ovary cells (CHO) or other recombinant human or humanized antibodies.
  • Patients who have taken Avastin ® intravenously in the 6 months prior to inclusion.
  • Patients who have had a therapeutic endoscopy for gastrointestinal bleeding or ENT surgery for epistaxis will have to wait at least 3 months less after treatment to be included if bleeding persists.
  • Patients who had a surgery in the month prior inclusion or planned surgery within 6 months
  • Severe peripheral arterial disease with ulcerations
  • Unhealed wound
  • Thrombosis in the 6 months prior to inclusion
  • Anticoagulant treatment
  • Uncontrolled high blood pressure
Read More

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Placebosodium chloride 0.9%0.9% of sodium chloride is infused every 14 days for 6 consecutive administrations
BevacizumabBevacizumabIntravenous infusion of Bevacizumab at a dose of 5 mg/kg
Primary Outcome Measures
NameTimeMethod
number of red blood cell transfusions6 months
Secondary Outcome Measures
NameTimeMethod
hemoglobin6 months

The relative evolution in hemoglobin level at 6 months after the beginning of the treatment is compared to the value measured at inclusion

epistaxis frequency3 months before treatment up to 6 months from the inclusion

Comparison of an average over a 3-month period before and after the treatment.

duration of nosebleeds3 months before treatment up to 6 months from the inclusion

Comparison of an average over a 3-month period before and after the treatment.

severity epistaxis score (ESS).6 months.

Comparison of ESS questionnaire before and after treatment

To evaluate pharmacokinetics of bevacizumab dose2 hours after the first treatment infusion

Description of bevacizumab serum concentrations over time, the relationship between bevacizumab concentrations and adverse events and clinical/biological endpoints

digestive vascular malformations6 months

Comparison of digestive endoscopy before and after treatment if gastrointestinal bleeding have already externalized before treatment

quality of life (SF36).6 months

Comparison of SF36 questionnaire before and after treatment.

adverse eventsup to 6 months

To assess the safety of bevacizumab.Tolerance will be evaluated by recording adverse events and by clinical examinations during the treatment period and the follow up period.

Trial Locations

Locations (4)

Hôpital Femme Mère Enfant

🇫🇷

Bron, France

Hôpital Ambroise Paré

🇫🇷

Boulogne Billancourt, France

CHU de Montpellier Hôpital St Eloi

🇫🇷

Montpellier, France

CHU d'Angers

🇫🇷

Angers, France

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