VWD Flashcards
How is VWD classified ?
❤Type 1 : partial quantitative
❤Type 2: qualitative
2B : ⬆️ affinity to plt ( complex VWF+plt ) 👉 ⬇️plt ( thrombocytopenia)
❤Type 3: severe quantitative
What is the inheritance of VWD ?
Type 1: autosomal dominant
Type 2: autosomal dominant
Type 3: autosomal recessive
What is the most common inherited bleeding disorder? What is the incidence among population ?
VWD
1/ 1000
What are the risks in pregnancy to mother with VWD ?
Increased risk of:
antepartum haemorrhage ( 10 fold)
Primary &secondary haemorrhage pph 25%
IN :
* type 1 whose VWF levels < 0.5
* type 2
* type 3
What is the variation in VWF in pregnancy of each type of VWD?
🔴Type 1: VWF levels rise progressively through pregnancy. ( usually adequate to rectify the deficiency)
🔴 type 2: may not correct or may worsen
🔴 type 3: minimal or no rise
What is the variation in VWF levels post delivery?
Start to fall : at around 3 days after delivery
Return to baseline: few days- several weeks .
What is the prepregnancy management in women with VWD?
1- bleeding phenotype should be assessed
2- response to DDAVP should be established.
3- iron deficiency should be corrected
4- screening for transfusion transmitted infections
5- vaccination: hepatitis A/B
🔴 tranexamic acid may continued
What is the antenatal management in women with VWD?
1- multidisciplinary team
2- VWF levels / activity +F8 levels:
* at booking
* 3rd trimester
* prior to any invasive procedure.
Except type 2B👉 monthly ( to assess the response to pregnancy)
What is the management when invasive procedures are required in women with VWD?
1- DDAVP ( in preference to factor concentrates) 👉 if (VWFa or F8)< 0.5
2- concentrate( F8+VWF): type 2B + type 3
❤ the target is :
VWF activity should be 1 iu and maintain above 0.5 until hemostasis is secured.
🔴 for most procedures: single preoperative treatment is sufficient
What cautions should be considered in women with VWD who are treated by DDAVP?
1- TYPE 2B : DON’T give DDAVP
( may cause thrombocytopenia)
2- fluids should be restricted to 1L 24 h
3- electrolytes should be monitored
( hyponatremia)
4- avoid DDAVP in women with preeclampsia
What is the optimal mode of delivery in women with VWD?
Spontaneous labour & vaginal delivery
What is intrapartum management for women with VWD?
🔴Type 1 + VWF activity > 0.5 👉 tranexamic acid as a sole therapy
( orally or IV )
🔴 type 1 + VWF activity < 0.5 👉 DDAVP or VWF concentrates with tranexamic acid
🔴type 2B 👉 VWF replacement + plt transfusion
❤ tranexamic acid dose : 1 g IV
What is the aim of treatment in women with VWD in intrapartum & postpartum period?
Intrapartum:
VWF activity & F8 1 - 1.5 iu / ml
Postpartum:
VWF activity & F8 0.5 - 1 iu/ ml
Is there any considerations about the fetuses at risk of having VWD during the labour?
Fetuses at risk of having VWD type 2 or type 3 : FBS + external cephalic version + fetal scalp monitoring + ventouse + midcavity forceps
SHOULD BE AVOIDED
How can analgesia & anaesthesia be safely managed in women with VWD?
❤Type 1 : VWF activity normalized by pregnancy 👉 central neuraxial anaesthesia ( CNA) can be offered
❤ type 2 CNA should be avoided unless VWF activity > 0.5
* In type 2N CNA should be avoidedUnless F8 > 0.5
🔴 if epidural catheter is placed 👉 repeat treatment before removal
🔴 avoid intramuscular injections
Or NSAIDS unless VWF activity + F8 >0.5
