What is GPS?
It is an inherited bleeding disorder associated by abnormal platelets which are small blood cells involved in blood clotting.
People with this condition tend to bruise easily and have increased risk of nosebleeds.
Signs and symptoms:
They usually appear at birth or in early childhood. These include low platelets counts, easy bruising and prolonged nosebleeds.
Female patients may have irregular and heavy menstrual periods (menometrorrhagia).
People with GPS may also experience abnormally heavy or extended bleeding from dental work, surgery, minor trauma or child birth.
The bleeding are usually mild to moderate but they have been life threatening in other individuals.
Myelofibrosis which is a condition characterised by a build-up of scar tissue in the bone marrow is a common feature of GPS
Gray Platelet Syndrome may result in fatal bleeding especially in adulthood when platelets counts are further decreased.
Affected people:
GPS is very rare about 60 cases have been reported worldwide
Treatment:
There is no specific treatment for GPS but management such as platelet transfusion before surgery is recommended.
Administration of desmopression should be considered to increase the platelet counts in people who have low platelets.