FranceCoag project
1. History
FranceCoag grew out of the French national haemophilia treatment cohort, the Suivi Thérapeutique National des Hémophiles (SNH), set up in 1994. Its initial purpose was pharmacovigilance: it was limited to haemophilia and focused on treated patients.1 The SNH comprised a database and a biobank. Collection took place over two periods (1994–2002 and 2008–2011) and stopped permanently in 2011.
In 2003, the SNH became the FranceCoag Network, with a broader epidemiological aim covering all inherited bleeding disorders caused by a hereditary coagulation protein deficiency. Coordinated first by Inserm and, from 2004, by the French Institute for Public Health Surveillance (InVS, now Santé publique France), the programme has been managed by Assistance Publique – Hôpitaux de Marseille (AP-HM) since January 2017, when it was renamed FranceCoag.2
2. Objectives
FranceCoag serves public-interest purposes:
- providing public authorities and patient associations with aggregated information on epidemiology and safety;
- providing data providers (institutional partners, including the MHEMO network) with information on the patients they follow and on their clinical practice;
- offering a secure database to public and private research organisations.
FranceCoag centralises, harmonises and archives, at national level, the personal data of patients with inherited bleeding disorders. Its main objectives are:
- Epidemiology: comprehensive knowledge of the geographical distribution, socio-demographic characteristics and changes over time of the population with inherited bleeding disorders;
- Health and drug safety surveillance: long-term identification and prevention of risks associated with exposure to high-risk treatments;
- Research: dynamic follow-up of a population of incident cases, and a database available for ancillary research projects, including linkage with the French National Health Data System (SNDS).
3. Target population
The following disorders are eligible for FranceCoag: haemophilia; severe forms of rare inherited coagulation factor deficiencies (afibrinogenaemia; deficiencies of factor II, V, VII, X, XI and XIII; combined factor V and VIII deficiency; combined deficiency of vitamin K-dependent factors); symptomatic forms of von Willebrand disease (VWD); and inherited platelet disorders.
FranceCoag operates as a registry, including all patients, for the most severe disorders:
- factor VIII or IX deficiency with a level ≤ 5% (severe or moderate haemophilia A and B, including haemophilia B Leyden with FIX ≤ 5%);
- the most severe forms of VWD, with VWF activity (VWF:Act) ≤ 15% or FVIII ≤ 5%;
- congenital afibrinogenaemia, with fibrinogen < 0.2 g/L;
- factor XIII deficiency with a level < 10%;
- Glanzmann thrombasthenia (GT);
- Bernard–Soulier syndrome (BSS);
- other registrable platelet disorders.
Follow-up in the cohort and its frequency are summarised below.
| Disorder | Cohort follow-up | Follow-up interval |
|---|---|---|
| Severe haemophilia A | All patients | Every year |
| Moderate haemophilia A | All patients | Every year |
| Mild haemophilia A | 1-in-10 sample | Every 4 years |
| Severe haemophilia B | All patients | Every year |
| Moderate haemophilia B | All patients | Every year |
| Mild haemophilia B | 1-in-10 sample | Every 4 years |
| Haemophilia B Leyden | All patients | Every year while FIX ≤ 5%, then every 4 years |
| Very severe VWD, types 3 and 2N (“haemophilia-like”): VWF < 5% or FVIII:C ≤ 5% | All patients | Every year |
| Severe VWD, types 1, 2A and 2M: 5% ≤ VWF ≤ 15% and FVIII:C > 5% | All patients | Every 2 years |
| Non-severe VWD, type 1 or 2: VWF > 15% and < 40%, or FVIII:C > 5% and < 40% | 1-in-10 sample | Every 4 years |
| Factor I deficiency | All patients | Every year |
| Factor XIII deficiency | All patients | Every year |
| Other rare deficiencies (excluding FI and FXIII) | All patients | Every 4 years |
| Registrable thrombocytopenias, including BSS | All patients | Every 2 years |
| Registrable platelet function disorders, including GT | All patients | Every 2 years |
| Non-registrable thrombocytopenias | No | Not applicable |
| Non-registrable platelet function disorders | No | Not applicable |
4. Data collected
Data are collected through seven main questionnaires: patient identification; general characteristics (diagnosis, demographics, family history, genetics); FranceCoag visits; comorbidities and events (joint and bleeding events, surgery, obstetric events, serious adverse events); treatment (replacement and non-replacement therapies); laboratory tests; and patient- or parent-reported data (education, employment, social situation, physical activity, health status and quality of life).
See the full list of data collected and the questionnaires
5. Protocol
FranceCoag operates under a protocol approved by the joint FranceCoag–MHEMO Scientific Committee. It sets out the objectives of FranceCoag, the eligible disorders, the inclusion and severity criteria, the dataset, the data collection methods and the procedures covering the whole data life cycle.
References
1. Calvez T, Biou M, Costagliola D, Jullien AM, Laurian Y, Rossi F, et al. The French haemophilia cohort: rationale and organization of a long-term national pharmacosurveillance system. Haemophilia. 2001;7(1):82-88.
2. Doncarli A, Demiguel V, Guseva Canu I, Goulet V, Bayart S, Calvez T, et al.; FranceCoag Network. FranceCoag: a 22-year prospective follow-up of the national French cohort of patients with inherited bleeding disorders. Eur J Epidemiol. 2019;34(5):521-532. doi:10.1007/s10654-018-0468-7
