Glanzmann ThrombastheniaTAASNCT04595617
Anti-αIIbβ3 Immunization in Glanzmann Thrombasthenia: Prevalence and Associated Risk Factors: Thrombasthenia Anti-αIIbβ3 Antibodies Study (TAAS)
A study testing Antibodies screening in people with Glanzmann Thrombasthenia, run by University Hospital, Bordeaux at 7 sites in France.
Trial summary
This project aim to correlate risk factors (genetic, therapeutic and socio-demographic factors) to anti-αIIbβ3 antibodies formation following blood products transfusion (platelets or packed red cells) or pregnancy in a national cohort of GT patients. Read more about this study
- Who is it for?
- Ages 6 years and older
- Conditions the study is for
- Glanzmann Thrombasthenia
- What is being tested?
- Antibodies screening
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 28 participants
- Where
- 7 sitesFrance
- Study dates
- 6 Jan 2021to 6 Jul 2023
- Phase
- Interventional
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 6 years and older
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
Who may be able to join
- All patients with a clear diagnosis of Glanzmann Thrombastenia (GT), whatever the subtype of disease.
- Affiliated person or beneficiary of a social security scheme.
- Free, informed and written consent signed by the participant, or parents or legal representant for the child population, and the investigator (at the latest on the day of inclusion and before any examination required by the research).
Who cannot join
- Current treatment that may interfere with anti-αIIbβ3 antibodies detection, such as intravenous immunoglobulins within the previous month.
- Psychiatric, social or behavioral condition judged to be non-compatible with the respect of the protocol, including good observance of treatment and compliance to follow-up.
- Adult protected by the law.
Trial locations and contacts
7 sites in 1 country.
7 sites in 1 country
France7 sites
CHU Bordeaux - Hôpital Haut-Lévêque
Bordeaux
CHU Bordeaux - Hôpital Pellegrin
Bordeaux
Hôpital Bicêtre, APHP
Le Kremlin-Bicêtre
Hôpital la Timone, APHM
Marseille
CHU Nîmes
Nîmes
CHU Strasbourg
Strasbourg
CHU Toulouse
Toulouse
About this study
This project aim to correlate risk factors (genetic, therapeutic and socio-demographic factors) to anti-αIIbβ3 antibodies formation following blood products transfusion (platelets or packed red cells) or pregnancy in a national cohort of GT patients.
Read the detailed description
Glanzmann thrombasthenia (GT) is a rare autosomal recessive disorder caused by the absence or the dysfunction of the αIIbβ3 integrin, the most abundant receptor on platelets that mediates platelet aggregation through its binding of adhesive proteins. GT is readily identifiable by platelet function testing, and a lack of platelet aggregation in response to all physiological agonists is unique for this disease. The ITGA2B gene encodes for the αIIb subunit, whereas the ITGB3 gene encodes for β3. Mutations causing GT can affect either ITGA2B or ITGB3. The disease is characterized by spontaneous and trauma-related mucocutaneous bleeding, with variable expression ranging from easy bruising to fatal hemorrhages. Platelet transfusions are used to control or prevent life-threatening blood loss, but can become ineffective due to naturally occurring antibodies directed against αIIbβ3. Such antibodies are produced when patient's immune system comes into contact with normal αIIbβ3 expressing platelets.
There is no currently consensus concerning the frequency, the long-term evolution, or the formation of characteristics of antibodies from GT patients in relation to the nature of the defective gene (ITGA2B or ITGB3), gene variations or other factors. Research are needed to confirm that nature of the gene defect may have a causative role in antibody development. Moreover, strength and persistence of antibodies may vary among patients with the same mutation, suggesting that other factors, such as immune modifiers genes, play a role in shaping antibody repertoire.
Monoclonal antibody-specific immobilization of platelet antigens (MAIPA) is still considered as the reference method for evaluating the presence of anti-αIIbβ3 antibodies in GT patients. All the tests will be performed by the principal investigator site (Bordeaux).
- Conditions
- Glanzmann Thrombasthenia
- Being tested
- Antibodies screening
- Keywords
- Glanzmann thrombasthenia, anti-GPIIb-IIIa immunization, MAIPA, Platelet transfusions, Recombinant activated factor VII
- Medical subject headings
- Thrombasthenia
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Antibodies screeningBiological
All included GT patients will be enrolled from different national centres during a 6 months period. Antibodies screening will be systematically realized every six months (+/- 2 weeks) and after each last blood transfusion at 7-10 days and one month (+/- 2 weeks), during a period of 18 months.
| Study group | Role | What they receive |
|---|---|---|
| Patients with diagnosis of Glanzmann Thrombastenia (GT) | Experimental | Antibodies screening will be systematically realized every six months (+/- 2 weeks) and after each last blood transfusion at 7-10 days and one month (+/- 2 weeks), during a period of 18 months Receives: Biological: Antibodies screening |
What is being measured
The primary outcome is the main question the study is designed to answer.
Characterization of change of an anti-αIIbβ3 immunization
Characterization of change of an anti-αIIbβ3 immunization will be assessed with Indirect MoAb-specific immobilization of platelet antigens (MAIPA)
Measured: From inclusion to 18 months visit
Number of patients with positive anti-αIIbβ3 antibodies in relation to risk factors
Risk factors could be subtype of GT, year of birth, ITGA2B or ITGB3 gene mutation
Measured: From inclusion to 18 months visit
Secondary outcomes (3)
Determination of the prevalence of anti-αIIbβ3 antibodies in a regional cohort of GT patients
Presence of anti-αIIbβ3 antibodies will be assessed with Indirect MoAb-specific immobilization of platelet antigens (MAIPA)
Measured: From inclusion to 18 months visit
Description of the kinetic of an anti-αIIbβ3 immunization following blood transfusion
Repetition of the antibodies measurements with Indirect MoAb-specific immobilization of platelet antigens (MAIPA)
Measured: At 7-10 days and 1 month (+/-2 weeks) after each blood transfusion
Determination of the mechanism of anti-αIIbβ3 antibodies blocking integrin function by determining the capacity of anti-αIIbβ3 antibodies to impair fibrinogen binding
In vitro studies will be performed by mixing serum of patients with washed donors' platelets and inhibition of the integrin will be studied by flow cytometry
Measured: through study completion, an average of 2 years
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted20 Oct 2020
- Study start6 Jan 2021
- Primary completion6 Jul 2023
- Study completion6 Jul 2023
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Glanzmann ThrombastheniaStudies of the same condition, open and under way.
- More trials run by University Hospital, BordeauxThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase N/A
- Primary purpose
- Diagnosis
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Enrolment
- 28 participants (actual)
Sponsor and investigators
- Lead sponsor
- University Hospital, Bordeaux
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Mathieu FIORE, Principal investigator, University Hospital, Bordeaux
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04595617
- Sponsor's protocol ID
- CHUBX 2019/41
- Other IDs
- 2020-A01285-34
- Acronym
- TAAS
- Status last verified
- Mar 2024
- First submitted
- 17 Aug 2020
- Last updated
- 22 May 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Study at a glance
From the registry record, last updated by the sponsor on 22 May 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Anti-αIIbβ3 Immunization in Glanzmann Thrombasthenia: Prevalence and Associated Risk Factors: Thrombasthenia Anti-αIIbβ3 Antibodies Study (TAAS)
- ClinicalTrials.gov ID
- NCT04595617
- Acronym / sponsor ID
- TAAS / CHUBX 2019/41
- Conditions
- Glanzmann Thrombasthenia
- Keywords
- Glanzmann thrombasthenia, anti-GPIIb-IIIa immunization, MAIPA, Platelet transfusions, Recombinant activated factor VII
- Study type
- Interventional
- Recruitment status
- Completed
- Enrolment
- 28 participants (actual)
- Lead sponsor
- University Hospital, BordeauxUniversity, hospital or other
- Study start
- 6 Jan 2021 (actual)
- Primary completion
- 6 Jul 2023 (actual)
- Study completion
- 6 Jul 2023 (actual)
- First posted
- 20 Oct 2020 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
This project aim to correlate risk factors (genetic, therapeutic and socio-demographic factors) to anti-αIIbβ3 antibodies formation following blood products transfusion (platelets or packed red cells) or pregnancy in a national cohort of GT patients.
Detailed description
Glanzmann thrombasthenia (GT) is a rare autosomal recessive disorder caused by the absence or the dysfunction of the αIIbβ3 integrin, the most abundant receptor on platelets that mediates platelet aggregation through its binding of adhesive proteins. GT is readily identifiable by platelet function testing, and a lack of platelet aggregation in response to all physiological agonists is unique for this disease. The ITGA2B gene encodes for the αIIb subunit, whereas the ITGB3 gene encodes for β3. Mutations causing GT can affect either ITGA2B or ITGB3. The disease is characterized by spontaneous and trauma-related mucocutaneous bleeding, with variable expression ranging from easy bruising to fatal hemorrhages. Platelet transfusions are used to control or prevent life-threatening blood loss, but can become ineffective due to naturally occurring antibodies directed against αIIbβ3. Such antibodies are produced when patient's immune system comes into contact with normal αIIbβ3 expressing platelets.
There is no currently consensus concerning the frequency, the long-term evolution, or the formation of characteristics of antibodies from GT patients in relation to the nature of the defective gene (ITGA2B or ITGB3), gene variations or other factors. Research are needed to confirm that nature of the gene defect may have a causative role in antibody development. Moreover, strength and persistence of antibodies may vary among patients with the same mutation, suggesting that other factors, such as immune modifiers genes, play a role in shaping antibody repertoire.
Monoclonal antibody-specific immobilization of platelet antigens (MAIPA) is still considered as the reference method for evaluating the presence of anti-αIIbβ3 antibodies in GT patients. All the tests will be performed by the principal investigator site (Bordeaux).
Study design
- Primary purpose
- Diagnosis
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Patients with diagnosis of Glanzmann Thrombastenia (GT) | Experimental | Biological: Antibodies screeningAntibodies screening will be systematically realized every six months (+/- 2 weeks) and after each last blood transfusion at 7-10 days and one month (+/- 2 weeks), during a period of 18 months |
Who may qualify
- Ages
- 6 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult, Older adult
Key inclusion criteria (3 listed)
- All patients with a clear diagnosis of Glanzmann Thrombastenia (GT), whatever the subtype of disease.
- Affiliated person or beneficiary of a social security scheme.
- Free, informed and written consent signed by the participant, or parents or legal representant for the child population, and the investigator (at the latest on the day of inclusion and before any examination required by the research).
Key exclusion criteria (3 listed)
- Current treatment that may interfere with anti-αIIbβ3 antibodies detection, such as intravenous immunoglobulins within the previous month.
- Psychiatric, social or behavioral condition judged to be non-compatible with the respect of the protocol, including good observance of treatment and compliance to follow-up.
- Adult protected by the law.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Antibodies screeningAll included GT patients will be enrolled from different national centres during a 6 months period. Antibodies screening will be systematically realized every six months (+/- 2 weeks) and after each last blood transfusion at 7-10 days and one month (+/- 2 weeks), during a period of 18 months. | Biological | Patients with diagnosis of Glanzmann Thrombastenia (GT) |
Primary outcome
Characterization of change of an anti-αIIbβ3 immunization
Time frame: From inclusion to 18 months visit
Characterization of change of an anti-αIIbβ3 immunization will be assessed with Indirect MoAb-specific immobilization of platelet antigens (MAIPA)
Number of patients with positive anti-αIIbβ3 antibodies in relation to risk factors
Time frame: From inclusion to 18 months visit
Risk factors could be subtype of GT, year of birth, ITGA2B or ITGB3 gene mutation
3 secondary outcomes
Determination of the prevalence of anti-αIIbβ3 antibodies in a regional cohort of GT patients
Time frame: From inclusion to 18 months visit
Description of the kinetic of an anti-αIIbβ3 immunization following blood transfusion
Time frame: At 7-10 days and 1 month (+/-2 weeks) after each blood transfusion
Determination of the mechanism of anti-αIIbβ3 antibodies blocking integrin function by determining the capacity of anti-αIIbβ3 antibodies to impair fibrinogen binding
Time frame: through study completion, an average of 2 years
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 22 May 2026.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Mathieu FIORE principal investigator
University Hospital, Bordeaux
7 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A study testing Antibodies screening in people with Glanzmann Thrombasthenia, run by University Hospital, Bordeaux at 7 sites in France.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 3 inclusion and 3 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 22 May 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 7 sites in France. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Characterization of change of an anti-αIIbβ3 immunization (time frame: From inclusion to 18 months visit). The study lists 2 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 22 May 2026.