Grey Platelet Syndrome

Grey Platelet Syndrome (GPS) is a rare, inherited platelet disorder named for the grayish appearance of the affected person's platelets when viewed with a microscope. Grey Platelet Syndrome is characterized by the deficiency of alpha-granules in the platelets, increasing the risk of bleeding disorders.

Genetics

• GPS is inherited from one parent who has an abnormality on one of the first 22 non-sex chromosomes. This mode of transmission is called autosomal inheritance.

Pathology

• The underlying problem concerning GPS is the inability of the body's megakaryocytes to properly pack protein into platelets, causing uncontrolled bleeding at the site of wounds. The proteins that should be distributed in the platelets are instead distributed in bone marrow, possibly causing myelofibrosis.

Symptoms

• Common symptoms of GPS include easy bruising, nosebleeds, menorrhagia, prolonged bleeding and a family history of bleeding.

Management

• Managing GPS involves anticipating bleeding and special considerations must be taken by your physician when surgery is required. It is not advisable to undergo plasma transfusion if you have GPS.

Prognosis

• GPS is not a life-threatening condition if all bleeding is kept under control. There have been no reported cases of fatal bleeds.