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© Kamla-Raj 2002 Int J Hum Genet, 2(3): 197-204 (2002)
Functional Status of Platelets and Hereditary Platelet Disorders
Dilip K. Bhattacharya
Department of Pathology, Vivekananda Institute of Medical Sciences, Ramakrishna Mission Seva Pratishthan, 99 Sarat Bose Road, Kolkata 700 026, West Bengal, India
KEY WORDS Platelet disorders; bone marrow; glycoproteins
ABSTRACT The circulating platelets are derived from bone marrow megakaryocytes in response to thrombopoitic growth factors. The clinical response with recombinant human thrombopoietin (Peg-VHUMGDF and Vh TPO) is being evaluated in chemotherapy induced thrombo-cytopenia. The pathophysiology of platelet adhesiveness in flowing blood is a complex process that involves fibronectin, collagen, platelet glycoproteins thrombo-spondin and a host of related components. Various inherited platelet disorders involve glycoproteins, Von Willebrand factor and release from platelet storage granules leading to von Willebrand disorder (vWD) thrombasthenia, Bernard Soulier disorder, macrothro-mbocytopenia, storage pool disorder and collagen receptor defect. The constituents released from adherent platelets modify the vessel tone vessel wall repair and coagulation.
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