It is also called as Bernard-Soulier syndrome. It usually occurs in newborn or early child hood. In this condition there will be lack of ability of the platelets to stick to the blood vessel which is injured resulting in abnormal bleeding.

It is an autosomal recessive disorder. It is due to deficiency of platelet gycoproteins. It is rare disoder.It may begin in infancy and continue throught out the life. Bleeding is precipitated by injury or surgery.

It is a congenital bleeding disorder charecterized by thrombocytopenia and large platelets

Symptoms

• Bruises.
• Nose bleeding or epistaxis.
• Gum bleeding.
• GI bleeding.

Diagnosis

• Platelet exmaintion: Platelets are few and large.
• Prolongesd bleeding time.

Treatment

Thereis no specific treatment, apart from platelet during bleeding episodes.