1. Treatment options in people with CBD according to the possibility of specifically correcting the haemostatic deficiency.
Bleeding Disorder | Specific replacement | Other haemostatic treatment |
Haemophilia A | FVIII concentrates* | |
Haemophilia B | FIX concentrates° | |
Haemophilia with inhibitors | ‐ | aPCC, rFVIIa |
Von Willebrand disease | Endogenous: DDAVP; Exogenous: VWF‐containing concentrates# | |
Fibrinogen deficiency | Fibrinogen concentrates^ | Cryoprecipitate, FFP |
FII deficiency | ‐ | PCC, FFP |
FV deficiency | ‐ | FFP |
FV+FVIII deficiency | ‐ | FFP |
FVII deficiency | FVII concentrates° | FFP, PCC |
FX deficiency | FX concentrate^ | PCC |
FXI deficiency | FXI concentrate^ | FFP, DDAVP |
FXIII deficiency | FXIII concentrates§ | FFP, cryoprecipitate |
VKD factor deficiency | ‐ | FFP, PCC |
Platelet function abnormalities | Platelet concentrates | DDAVP, rFVIIa |
Fibrinolysis abnormalities | ‐ | FFP, antifibrinolytics |
aPCC: activated prothrombin complex concentrate DDAVP: desmopressin FFP: fresh frozen plasma PCC: prothrombin complex concentrates rFVIIa: activated recombinant factor VII VKD: vitamin K‐dependent VWF: von Willebrand factor
*Various intermediate‐ and high‐purity plasma‐derived concentrates and recombinant products available °Various high‐purity plasma‐derived concentrates and a single recombinant product currently available ^Plasma‐derived concentrate, not licensed in all countries #A series of plasma‐derived intermediate‐ and high‐purity FVIII concentrates, with different VWF/FVIII ratio, and a single concentrate of virtually VWF alone available
§ One plasma‐derived concentrate and a recently developed recombinant FXIII (A subunit) product, not available in all countries