Actively Recruiting
Immunological Effects of Iron Supplementation in HHT Disease
Led by Hospices Civils de Lyon · Updated on 2025-10-03
155
Participants Needed
1
Research Sites
170 weeks
Total Duration
On this page
AI-Summary
What this Trial Is About
Hereditary haemorrhagic telangiectasia (HHT), is a rare genetic vascular disorder with autosomal dominant inheritance. Its prevalence is estimated at approximately 1 in 6,000 individuals in France. Clinical manifestations include recurrent nosebleeds (epistaxis), cutaneous telangiectasias, and visceral arteriovenous malformations (AVMs) that may affect the lungs, gastrointestinal tract, liver, and brain. Beyond vascular abnormalities, patients often present with a decrease in circulating T lymphocytes (T-cell lymphopenia), which can be profound but remains unexplained. There is also a distinct infectious risk profile associated with the disease: brain abscesses in the presence of pulmonary AVMs (pAVMs), and osteoarticular infections in patients with the longest durations of epistaxis. However, no definitive correlation has been established between T-cell lymphopenia and infection risk. Iron-deficiency anemia is a frequent complication in HHT, affecting about 50% of patients, with a mean age of onset around 36 years. Its prevalence increases with age. These patients typically require prolonged and high-dose iron supplementation, administered either orally or intravenously, which may expose them to side effects not observed in other clinical contexts. In a previous study, we identified a correlation between the level of iron supplementation (none, oral, or intravenous) and the severity of T-cell lymphopenia. This association may be explained by two potential mechanisms linking iron metabolism to immune function: * A direct toxic effect of iron on immune system homeostasis * Impaired lymphocyte production resulting from iron deficiency, with the type of supplementation serving as an indirect marker of deficiency severity We propose a prospective study designed to differentiate between these two hypotheses. The aim of the study is to characterize the impact of iron deficiency and iron supplementation on the immune system of patients with HHT.
CONDITIONS
Official Title
Immunological Effects of Iron Supplementation in HHT Disease
Who Can Participate
Eligibility Criteria
You may qualify if you...
- Adult patient diagnosed with HHT meeting 3 or 4 Curaao criteria
- Documented pathogenic mutation in ENG, ACVRL1, or MADH4 genes
- Patient enrolled in the CIROCO cohort
- Written informed consent given and signed
- Covered by a social security scheme or equivalent
- Routine biological follow-up for HHT within 15 days before inclusion (blood count, reticulocytes, ferritin, CRP, calcium, phosphorus)
- Group 1: Ferritin > 25 g/L, no iron supplementation or red blood cell transfusion in past 3 months
- Group 2: Ferritin > 25 g/L, ongoing oral iron therapy or at least one intravenous iron infusion or red blood cell transfusion in past 3 months
- Group 3: Ferritin < 25 g/L, no iron supplementation or red blood cell transfusion in past 3 months
You will not qualify if you...
- Hemoglobin levels below 90 g/L
- Active cancer or cancer remission less than 3 months ago
- Active infection or infection recovery less than 3 months ago
- Autoimmune or autoinflammatory disease active or recently treated within 3 months requiring immunosuppressive therapy
- Pregnant, postpartum, or breastfeeding women
- Minors
- Individuals deprived of liberty by judicial or administrative decision
- Individuals under psychiatric care
- Individuals admitted to healthcare or social institution for reasons other than research participation
- Adults under legal protection (guardianship, curatorship)
- Participation in another interventional clinical trial with exclusion period still active
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Trial Site Locations
Total: 1 location
1
Hôpital Femme-Mère-Enfant
Bron, Rhone, France, 69500
Actively Recruiting
Research Team
A
Alexandre Guilhem, MD
CONTACT
How is the study designed?
Study Type
INTERVENTIONAL
Masking
NONE
Allocation
NON_RANDOMIZED
Model
PARALLEL
Primary Purpose
HEALTH_SERVICES_RESEARCH
Number of Arms
3
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