Czech National Haemophilia Programme (CNHP)

The CNHP registry – a registry of haemorrhagic conditions within the Czech National Haemophilia Programme (CNHP) – has been created in order to keep records on patients with inherited haemorrhagic conditions: haemophilia, von Willebrand's disese and other congenital haemorrhagic coagulopathies. The CNHP registry was established in May 2011 as a continuation of the HemIS registry (the primary aim of which was to monitor the numbers of haemophilia patients).

Haemophilia is a group of hereditary genetic disorders that impair the body's ability to control blood clotting or coagulation. Like most recessive sex-linked, X chromosome disorders, haemophilia is more likely to occur in males than females. Females are almost exclusively asymptomatic carriers of the disorder.

Von Willebrand's disease is the most common hereditary coagulation abnormality. It arises from a deficiency or abnormal structure of von Willebrand factor (vWF). There are three types of hereditary vWD, (Type I, Type II Type III). Within the three inherited types of vWD, there are various subtypes. The condition affects both men and women.

Other congenital haemorrhagic conditions are very rare, usually arising from decreased activity of a coagulation factor or dysproteinaemia. These inherited conditions are mostly autosomally recessive. The bleeding severity depends on how much the coagulation activity of a factor has been affected; in less severe forms, unusual bleeding can be discovered during a surgical intervention or after an injury.

Primary aim of the project:

• To keep records on patients suffering from congenital haemorrhagic conditions, the severity of their condition and the usage of medicinal products.

Secondary aims of the project:

• To monitor the numbers of bleeding episodes and of adverse drug reactions.
• Quality of life assessment.

Other important information:

• Expert guarantor: Associate Professor Jan Blatný, Ph.D., MD
• Partners: a network of specialised CCCs (Comprehensive Care Centres) and HTCs (Haemophilia Treatment Centres) for adult and paediatric patients (you can find more detailed information – in Czech language only – on the website of the CNHP project)
• Brief overview of results: you can find more detailed information – in Czech language only – on the website of the CNHP project