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Glanzmann Thrombasthenia is
a rare inherited bleeding disorder caused by a defect in platelet
aggregation. Platelets are blood cells that help stop
bleeding by sticking together at sites of blood vessel injury. In this
condition, platelets are present in normal or near-normal numbers but cannot
aggregate effectively because of abnormalities in the glycoprotein IIb/IIIa
receptor on their surface.
The disorder is usually
inherited in an autosomal recessive pattern, meaning an affected
individual typically receives an altered gene from both parents. Symptoms often
appear during childhood and may include frequent nosebleeds, easy bruising,
bleeding from the gums, heavy menstrual bleeding, prolonged bleeding from minor
cuts, and excessive bleeding after surgery or dental procedures. The
severity can vary between individuals.
Diagnosis may involve a complete
blood count, platelet function testing, and specialized laboratory studies
to identify impaired platelet aggregation. Genetic testing can help confirm the
underlying cause.
Treatment focuses on
controlling and preventing bleeding. Local measures may be sufficient for minor
episodes, while antifibrinolytic medicines or platelet transfusions may
be required for significant bleeding or surgical procedures. In selected severe
cases, recombinant factor VIIa may be considered. Careful planning with a
haematologist is important for long-term management.