Background (RBDs)
- Rare bleeding disorders (RBDs) include deficiencies of factors I (Fibrinogen), II, V, VII, X, XI and XIII
- Severe (similar to haemophilia) or mild
- Expert advice from a CCC is always required
Severity
Severity relates to the baseline level of the deficient factor but in some deficiencies, the patient's personal bleeding history may need to be considered. The patient's CCC will be able to advise on the bleeding severity for an individual patient.
More on National treatment guidelines (CUH Intranet).
Treatment options (if indicated)
- Fibrinogen: Riastap (Fibrinogen concentrate)
- Factor II: Octaplex (PCC)
- Factor V: Octaplas (SD plasma)
- Factor VII: NovoSeven (Recombinant factor VIIa)
- Factor X: Coagadex (Factor X Concentrate))
- Factor XI: Octaplas (SD plasma)
- Factor XIII: Fibrogammin P (FXIII concentrate)
- In all cases, please involve Haematology before treating