REVIEW ARTICLE Inherited platelet-based bleeding disorders

Summary. Inherited platelet-based bleeding disorders include abnormalities of platelet number and function, and are generally classified based on the abnormal functions or responses. How-ever, a clear distinction is problematic, and in this review, the classification has been based on abnormalities of platelet com-ponents that share common characteristics. Inherited thrombocytopenias are rare, but probably under-diagnosed. They are usually classified according to both platelet size and the presence or absence of clinical features other than those deriving from the platelet defect. Hereditary disorders of platelet function can be classified as resulting from: (i) abnorm-alities of the platelet receptors for adhesive proteins; (ii) abnormalities of the platelet receptors for soluble agonists; (iii) abnormalities of the platelet granules; (iv) abnormalities of the signal-transduction pathways; (v) abnormalities of the membrane phospholipids; and (vi) miscellaneous abnormalities of platelet function. The literature on these disorders is re-viewed, and the underlying defects discussed