Platelet Function Disorder

Platelets are very small cells that are an important part of the clotting process to stop bleeding. When a blood vessel is damaged, platelets along with Von Willebrand factor go to the damaged area to form a plug to stop the bleeding. When the platelets are actively forming a blood clot, they send out a chemical that calls for other platelets to help form a plug.

The normal amount of platelets in a person’s body is between 150,000 and 350,000. When an individual has less than the normal amount of platelets, they have a platelet disorder called thrombocytopenia, and it is important that consultation with a hematologist be done to figure out why the platelet number is low.

Sometimes, a patient can have a normal number of platelets, but the platelets don’t work well - these are called Platelet Function Disorders, or sometimes just Platelet Disorders.

Platelet Function Disorders are divided into 3 categories:

  • Adhesion Disorders
  • Aggregation Disorders
  • Secretion Disorders

Adhesion Disorders

An adhesion disorder is the result of the platelets not being able to stick to the wall of the damaged blood vessel to form a plug and stop the bleeding.

Bernard-Soulier Syndrome

Bernard-Soulier Syndrome is a rare disorder that is inherited or passed down from a child’s parent(s). A child with Bernard-Soulier Syndrome has platelets that are large and missing a chemical that helps the platelets stick to the wall of a damaged blood vessel. Symptoms with this disorder range from mild to severe:

  • Bleeding that does not stop for 10 to 20 minutes
  • Mouth, dental or nose bleeding
  • Bleeding for a long time after surgery or an injury
  • Gastro-intestinal bleeding (Blood in vomit or stool)
  • Women may have heavy menstrual periods or bleeding after child birth

TREATMENT:

  • Avoid aspirin or medicines affecting platelet function
  • Avoid activities or sports where there is a higher risk of injury 
  • Tell other health care providers that you or your child has a platelet disorder
  • Girls or women may need hormonal therapy to control their heavy periods
  • Epistaxis care for a nosebleed
  • Stimate (intranasal DDAVP) may be used in certain platelet disorders

Aggregation Disorders

An aggregation disorder is when platelets do not bind with fibrinogen and other proteins in order to stick to other platelets. As a result, the platelets cannot form a plug to stop the bleeding from a damaged blood vessel.

Glanzmann’s Thromboasthenia

Glanzmann’s Thromboasthenia is a disorder that is inherited or passed down from a child's parent(s). Glanzmann's Thromboasthenia causes moderate to severe bleeding symptoms:

  • Bleeding from the mouth
  • Bleeding with dental procedures
  • Nose bleeds
  • Bruising or small purplish red dots under the skin
  • Bleeding for a long time after an injury or surgery
  • Girls or women may have heavy periods
  • Infant boys may have bleeding after circumcision

Other Aggregation Disorders

People who have other aggregation disorders have a low amount or are missing chemicals needed to help platelets stick together. If the platelets cannot stick together, then they cannot form a plug to stop the bleeding from a damaged blood vessel.

TREATMENT:

  • Avoid aspirin or medications affecting platelet function
  • Avoid activities or sports where there is a higher risk of injury
  • Tell other health care providers that you or your child has a platelet disorder
  • Girls or women may need hormonal therapy to control their heavy periods
  • Epistaxis care for a nosebleed 
  • Stimate (intranasal DDAVP) may be used in certain platelet disorders

Secretion Disorders

A secretion disorder is when the damaged blood vessel takes more time for the bleeding to stop due to missing chemicals that signals the platelets to stick together. As a result, it takes a lot longer for the bleeding from a damaged blood vessel to stop. This is the most common platelet disorder.