Platelet Dysfunction

Platelets are essential to primary and secondary hemostasis, including coagulation, or clot formation. When platelets do not function properly, prolonged bleeding may occur. Bleeding complications in inherited platelet disorders illustrate the importance of platelets to normal hemostasis. Causes of inherited platelet dysfunction range from defects in receptors important for platelet adhesion and aggregation (GPIb, GPIIb/IIIa) to defects in signaling molecules or transcription factors. The defect might also affect platelet activation, secretion and the secondary wave of platelet aggregation.

Platelet Disorder Symptoms

Platelet-type bleeding symptoms include mucocutaneous bleeding, such as easy bruising, petechial bleeding, epistaxis and excessive bleeding following invasive surgical and dental procedures. However, there is considerable heterogeneity in the severity of bleeding problems associated with congenital platelet disorders. Congenital platelet disorders can alter circulating platelet number, function or both. Some of the more common inherited platelet disorders include Bernard-Soulier-Syndrome, Glanzmann thrombasthenia, storage pool disorders and Wiskott-Aldrich-Syndrome.

Symptoms of a platelet disorder include:

• Bruising
• Nosebleeds (epistaxis)
• Mucocutaneous and other petechial bleeding
• Menorrhagia
• Bleeding after surgical and dental procedures such as tonsillectomy, adenoidectomy, tooth extraction and childbirth