Accueil Presentation of France COAG FranceCoag project Target population
The diseases eligible for the FranceCoag scheme are haemophilia, severe forms of rare HCM caused by another inherited coagulation protein deficiency (HCPD: afibrinogenemia, FII, FV, FVII, FX, FXI and FXIII deficiencies, FV+VIII, combined vitamin K-dependent factor deficiencies), symptomatic forms of Willebrand’s disease (WM) and inherited platelet diseases.
The rate of monitoring and the size of the populations monitored are described in the table below. The FC acts as a registry for the most severe pathologies, i.e. :
- FVIII and FIX deficiencies with levels ≤ 5% (severe or moderate haemophilia A and B, including Leyden B forms with FIX ≤ 5%);
- the most severe forms of WM with a Willebrand Factor activity level (vWF:Act) ≤ 15% or an FVIII level ≤ 5% ;
- congenital afibrinogenemia with a fibrinogen level < of 0.2 g/l ;
- FXIII deficiency with an < rate of 10% ;
- Glanzmann thrombasthenia (TG);
- Jean Bernard Soulier syndromes (SBS) ;
- other registrable platelet diseases.
| MHC | Cohort tracking | Cohort follow-up rate |
|---|
| Severe haemophilia A | All | Every year |
| Moderate haemophilia A | All | Every year |
| Haemophilia A minor | 1/10th sample | Every 4 years |
| Severe haemophilia B | All | Every year |
| Hémophilie B modérée | All | Every year |
| Haemophilia B minor | 1/10th sample | Every 4 years |
| Haemophilia B Leyden | All | Annual as long as FIX rate ≤ 5%, then every 4 years |
| Very severe WM type 3 and 2N ("haemophilia-like") VWF level <5% OR FVIII:C ≤ 5%. | All | Every year |
| Severe WM types 1, 2A, 2M 5% ≤ VWF level ≤ 15% and an FVIII:C level> 5% and< 40%.
| All | Every 2 years |
| WM other than severe type 1 or 2 : VWF level > 15% and< 40% OR an FVIII:C level > 5% and <40%. | 1/10th sample | Every 4 years |
| FI | All | Every year |
| FXIII | All | Every year |
| Rare deficiencies other than IF and FXIII deficiencies | All | Every 4 years |
| Registrable thrombocytopenia including BSS | All | Every 2 years |
| Registrable thrombopathies including TG | All | Every 2 years |
| Non-registrable thrombocytopenia | No | Not applicable |
| Non-registerable thrombopathies | No | Not applicable |
