Platelet Function Disorders

Question 1

What is the aetiology of Bernard Soulier Syndrome BSS?

Answer 1

Genetic abnormality of platelet adhesion receptor Gp1b
👉 abnormal adhesion of platelets
👉 normal plt count + ⬆️ Bt

Question 2

What is the inheritance of Bernard Soulier Syndrome BSS?

Answer 2

Autosomal recessive

Question 3

What are the maternal risks in women with Bernard Soulier syndrome BSS?

Answer 3

1-Significant risk of primary &secondary PPH
2- wound hematoma

Question 4

What is the management of delivery in women with Bernard Soulier syndrome BSS?

Answer 4

1- multidisciplinary team
2- platelets transfusion prophylactically before CS or vaginal birth
3- tranexamic acid: at the onset of the labour and continued through postpartum until lochia is minimal
4- CNA : should be avoided

Question 5

What is the aetiology of Glanzmann’s thrombasthenia GT?

Answer 5

Disorder of platelets function
Caused by lack of Gp2b/3a 👉 impaired plt-plt aggregation
* normal plt count
* ⬆️ Bt

Question 6

What is the inheritance of Glanzmann’s thrombasthenia GT?

Answer 6

Autosomal recessive

Question 7

What are the maternal risks in women with Glanzmann’s thrombasthenia GT?

Answer 7

1- intrapartum haemorrhage
2- PPH

Question 8

What is the management of delivery in women with Glanzmann’s thrombasthenia GT?

Answer 8

❤ At delivery:
Prophylactic platelet transfusion
Or
Activated Factor 7
❤ tranexamic acid should be given from the onset of the labour until the lochia is minimal
❤ CNA should be avoided

Question 9

What are the risks to the fetus in women with Glanzmann’s thrombasthenia GT?

Answer 9

Maternal alloimmunisation to fetal Gp2b/3a 👉 fetal thrombocytopenia
⬆️ risk of ICH

Question 10

What is the antenatal management in women with Glanzmann’s thrombasthenia GT?

Answer 10

1- platelet specific alloantibodies should be monitored:
- at booking
- 28 weeks
- 34 weeks
2- if fetal maternal alloimmunisation occurs:
- IV immunoglobulin
- steroids