Fibrinogen Disorders

Question 1

What are the functions of fibrinogen?

Answer 1

Hemostasis ( coagulation 1+2)
Fibrinolysis
Wound healing
Placental implantation
Acute phase reactant APR

Question 2

What is the normal range of fibrinogen?

Answer 2

200- 400 mg/dl

Question 3

What is the inheritance of afibrinogenaemia
Hypofibrinogenaemia
Dysfibrinogenaemia ?

Answer 3

afibrinogenaemia AR
Hypofibrinogenaemia AD
Dysfibrinogenaemia AD

Question 4

What are the risks in pregnancy to mother and baby in fibrinogen disorders?

Answer 4

Severe deficiency 👉 bleeding
Qualitative &qualitative deficiency can be associated with 👉 thrombosis +pregnancy loss

Question 5

What is the management of pregnancy and delivery in women with fibrinogen disorders?

Answer 5

*Pregnancy: fb< 50 👉 fb concentrate/ to maintain fib > 100
* labour: maintain fb > 150 for at least 3 days
* tranexamic acid can be used for minor bleeding.
❤ AVOID: CNA + NSAIDS + intramuscular injections
❤ AVOID: midcavity forceps + ventouse + fbs + FSE in a baby at risk for fibrinogen deficiency.

Question 6

What is the major risk of fibrinogen replacement therapy?

Answer 6

Thrombosis
Thrombotic phenotype/ other risk factors for venous thromboembolism 👉 LMWH