Glanzmann's Thrombasthenia

Question 1

What is Glanzmann’s Thrombasthenia and how does it arise?

Answer 1

• A platelet disorder which leads to increased bleeding.
• Due to mutations leading to a defect in the GPIIaGPIIIa complex on platelets, which is involved in binding to fibrinogen in primary haemostasis.

Question 2

What are the symptoms and their explanations?

Answer 2

• Prolonged bleeding time, nose bleeds, bleeding gums, heavy periods – inability of platelets binding fibrinogen to form a stable plug.
• Extensive bruising/purpura - loss of effective clotting forms massive haematomas and bruising.

Question 3

What are the laboratory findings?

Answer 3

• Normal platelet count and morphology

- Prolonged PT, APTT, TT

Question 4

What further tests should be carried out?

Answer 4

• PFA (platelet function) - decreased

- Failure to aggregate with ADP and collagen, but will aggregate with risocetin

Question 5

How is GT treated?

Answer 5

• Generic pressure, cauterisation and ice packs to control the bleeding
• Thrombomimetics – increase platelet production
• Platelet transfusion – counteracts abnormal platelet function