Table 1.

Classification of inherited disorders of platelet function

Disorder	Type	Abnormality
Disorders of aggregation (defects in platelet-platelet interaction)	Glanzmann thrombasthenia	deficiency or defect in GP Ilb-IIIa
Disorders of adhesion (defects in platelet-vessel wall interaction)	Bernard-Soulier syndrome platelet-type von Willebrand disease	deficiency or defect in GP Ib-IX-V
Disorders of platelet secretion (primary secretion defects) and signal transduction	defects in platelet-agonist interactions abnormalities in arachidonic acid path-ways or thromboxane A2 synthesis defects in G-protein activation defects in phosphatidylinositol meta-bolism defects in protein phosphorylation defects in calcium mobilization	deficiencies of receptors for ADP, collagen, epinephrine or thromboxane A2 impaired liberation of arachidonic acid, cyclooxygenase deficiency or thromboxane synthase deficiency Gαq deficiency phospholipase C-β2 deficiency PKC deficiency (Pleckstrin)
Disorders of platelet secretion and abnormalities of granules	storage pool diseases (8, a, a8)	8-granules, α-granules or both
Disorders of procoagulant function	Scott syndrome	membrane phospholipid defects
Defects in structural or cytoskeletal components	MYH9-related disorders Wiskott-Aldrich syndrome