What Are Giant Platelets?
Platelets that are larger than usual, up to 10 percent larger than normal, are considered giant or squashed platelets. Normal sized platelets range from 1.5 to 3.0 micrometers or µm in diameter and giant platelets, larger then 7 µm, range from 10 to 20 µm in diameter. Giant platelet syndrome also occurs in the Bernard-Soulier syndrome, a rare genetic disorder found in patients with these platelets. These abnormal platelets interfere with the clotting process, resulting in bleeding disorders and prolonged bleeding episodes.-
Bernard-Soulier Syndrome
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Bernard-Soulier Syndrome or B-SS, usually occurs in infancy or early childhood. A rare, genetic bleeding disorder caused by unusually shaped or giant platelets, the syndrome prevents clotting as the platelets lack the ability to adhere to the injured walls of blood vessels, a vital aspect in the forming of a clot at the wound site. This abnormality results in excessive bleeding.
Symptoms
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The syndrome usually begins in infancy or early childhood and causes nosebleeds, bleeding gums and in some cases, bruising. Problems that occur later include those associated with menstruation, surgery, stomach ulcers and trauma. The syndrome is usually suspected early, as children with the syndrome have problems with prolonged bleeding.
Causes
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The gene that carries the syndrome is inherited and transmits in the "autosomal recessive pattern," which means it is rare and requires both parents' contribution of the gene to the child. According to an article by David Perlstein, M.D., the syndrome is believed to have occurred in less than one in 1 million individuals and is due to a defect in the platelet glycoprotein.
Diagnosis
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Diagnosis of B-SS generally occurs after the patient seeks treatment for bleeding episodes. Infant boys with excessive bleeding after circumcision or older children experiencing prolonged bleeding after losing a tooth are most often expected of having giant platelet syndrome or B-SS. A special laboratory test confirms the presence of the disease.
Treatment
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There is no cure for giant platelet syndrome or B-SS, but studies are ongoing. In addition, there are no specific treatments for the disease. Patients diagnosed with B-SS must avoid aspirin, ibuprofen, naproxen or any medications that interfere with clot formation. If the patient experiences a severe bleeding episode, blood transfusions are required. Doctors recommend diagnosed patients to avoid all contact sports. Because anemia is a concern for those with B-SS, a daily iron supplement is advised as well.
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