Clinical disorders - Secondary hemostasis - Hemophilia
12 important questions on Clinical disorders - Secondary hemostasis - Hemophilia
What is the definition of hemophilia A and B?
What is the classification of severity in hemophilia A and B patients?
- Severe hemophilia: <1% active factor
- Moderate hemophilia: <5% active factor
- Mild hemophilia: 5-50% active factor
What are the symptoms of hemophilia?
- Muscle and joint bleeds
- Bleeds after trauma and surgery
- Bleeds starts several hours after trauma
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What are the genetics principles of hemophilia?
What are the 3 main treatment options for hemophilia and when do you use each medicine?
- Tranexaminic acid: when the patient has mucosal bleeds.
- DDAVP: patients with mild hemophilia A!
- Coagulation factor concentrate from plasma or recombinant
- Hemophilia A --> supplementation FVIII
- Hemophilia B --> supplementation FIX
What is the half-life time of supplementation FVIII and FIX
FIX: 24 hours
What is the effect of administration of the coagulation factor for hemophilia A and B?
The effect of administration of the coagulation factor is:
- 1 unit FVIII/kg increases FVIII with 2%
- 1 unit FIX/kg increases FIX with 1%
What is the aim for the on-demand treatment when a patients needs surgery?
The following days after the surgery, the factor levels needs to be:
- Day 1: 80-100%
- Day 2-5: 50-80%
- Day 3: 30-50%
What dosage of prophylaxes is given in patients with hemophilia A and B?
- Hemophilia A: 3x/week 20-40 EH/kg with extended half-life product 2x/week 20-40 EH/kg
- Hemophilia B: 2x/week 30-50 EH/kg with extended half-life product 1x/week 30-50 EH/kg
What are the 4 major complications of hemophilia treatment?
- Infection agents: only in plasma, infections like hepatitis A,B,C, HIV, parvocirus and Creatzfield Jacob
- Inhibitor development: development of alloantibodies directed against FVIII or FIX, which can lead to anaphylaxis or nephrotic syndrome.
- Thrombosis: when you give too much factor concentrate or when the patient has a disease that already increases the risk of thrombosis.
- Allergic reaction: to the factor concentrate
What do you measure when there is inhibitor development and how do you treat this?
Low titer <5 BU
- treatment: high dose of factor concentrate
High titer >5 BU
- Bypassing agents: Feiba (activated prothrombin complex concentrate) or recombinant FVII
- Immune tolerance induction: high dose of FVIII/FIX with corticosteroïds
What are the risk factors of forming inhibitors against FVIII and FIX?
- Race
- Age
- Family history
- Start of prophylaxes, dosing, type of factor product
- Type of mutation (intron 22 for FVIII and large gene deletion for FIX)
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