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Prospective Descriptive Study of the Angiogenic T Cell Population in Subjects With Hereditary Hemorrhagic Telangiectasia (HHT)

NCT ID: NCT03572556

Last Updated: 2020-11-20

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

60 participants

Study Classification

OBSERVATIONAL

Study Start Date

2018-06-28

Study Completion Date

2020-05-03

Brief Summary

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Hereditary hemorrhagic telangiectasia (HHT) results from genetic deregulation of angiogenesis. It is characterized by mucocutaneous telangiectasia responsible for recurrent epistaxis affecting quality of life (anaemia, iron deficiency, social distress). More rarely, HHT is complicated by the appearance of pulmonary, hepatic or cerebral arteriovenous malformations that can lead to serious complications: cerebrovascular accidents, cerebral abscesses, high output heart failure, and massive hemoptysis (1). The intensity of symptoms increases with age but with significant individual variability, even for the same mutation in the same family. Thus, while the mutations responsible for the disease have been identified, the pathophysiology is not fully understood because these mutations do not explain the great diversity of clinical presentations. Other factors not yet identified probably play an important role. Angiogenic T cells (TANG) are a newly individualized T cell population, defined by a CD4+CXCR4+CD31+ phenotype, which plays a key role in differentiating endothelial progenitors (2).

In an earlier study, the investigators showed that patients with HHT had a decrease in CD4+ and CD8+ LT compared to a cohort of healthy subjects (3).

They hypothesize that the lymphopenia mainly involves TANG, whose quantification could make it possible to assess the individual level of angiogenesis during HHT. The evaluation of the TANG levels could thus make it possible to personalize HHT management.

Detailed Description

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Conditions

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Hereditary Hemorrhagic Telangiectasia

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Patients

Hereditary hemorrhagic telangiectasia patients

Blood samples

Intervention Type BIOLOGICAL

* 5 mL dry tube to separate serum
* Two 6 mL EDTA tubes for plasma separation
* Eight 6 mL heparinized tubes for flow cytometry (quantification of TANG such as CD3+CD31+CXCR4+ and CEC) and quantification of angiogenesis markers.

Epistaxis charts

Intervention Type OTHER

Three monthly epistaxis charts to be completed

Controls

Matched for age (+/- 5 ans) and sex.

Blood samples

Intervention Type BIOLOGICAL

* 5 mL dry tube to separate serum
* Two 6 mL EDTA tubes for plasma separation
* Eight 6 mL heparinized tubes for flow cytometry (quantification of TANG such as CD3+CD31+CXCR4+ and CEC) and quantification of angiogenesis markers.

Interventions

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Blood samples

* 5 mL dry tube to separate serum
* Two 6 mL EDTA tubes for plasma separation
* Eight 6 mL heparinized tubes for flow cytometry (quantification of TANG such as CD3+CD31+CXCR4+ and CEC) and quantification of angiogenesis markers.

Intervention Type BIOLOGICAL

Epistaxis charts

Three monthly epistaxis charts to be completed

Intervention Type OTHER

Eligibility Criteria

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Inclusion Criteria

* Person who has given consent
* Adult
* Person capable of understanding spoken and written French

"Patient" group:

* Certain HHT (3 or 4 Curacao criteria - Appendix 2):
* Recurring epistaxis
* Telangiectasia of the skin or mouth
* Family hereditary context
* Arteriovenous visceral malformations
* Causal mutation identified
* Person capable of completing monthly epistaxis charts

"Control" group :

\- Control subjects will be matched to patients for age (+/- 6 years) and sex.

Exclusion Criteria

* Person not affiliated to a national health insurance scheme
* Pregnant or breastfeeding woman
* Protected adult
* Hemoglobin levels less than 9 g/dl in the last 15 days
* Progressive or recent infectious disease, autoimmune disease or cancer (less than 6 months)
* Immunosuppressive treatment in progress or recent (less than 6 months), including systemic steroid therapy. The use of inhaled or topical steroids is not an exclusion criterion.
* Treatment in progress or stopped less than 6 months ago or to be introduced within the next 3 months of the following medications:

* bevacizumab
* tranexamic acid
* dipeptidyl peptidase 4 inhibitors (diabetic patient)
* beta-blockers (hypertensive patient)
Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Centre Hospitalier Universitaire Dijon

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Locations

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CHU Dijon Bourgogne

Dijon, , France

Site Status

Countries

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France

Other Identifiers

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GUILHEM AMRO/AOI 2017

Identifier Type: -

Identifier Source: org_study_id