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Chapter 9
Disorders of platelet number and function
Platelet biology: structure and function
Hemostasis encompasses a series of interrelated and simultaneously occurring events involving the blood vessels, platelets, and coagulation system. Defects affecting any of these major participants may lead to a hemostatic defect and a bleeding disorder. This chapter will focus on the disorders related to platelet number and function.
Platelet structure
Blood platelets are anucleate fragments derived from bone marrow megakaryocytes. The mean diameter of platelets ranges from approximately 1.5 to 3.0 µm, roughly one third to one fourth that of erythrocytes. Platelet volume is approximately 7 fL. Electron microscopy reveals a fuzzy coat (glycocalix) on the platelet surface composed of membrane glycoproteins, Platelet function in hemostasis
Regulation of platelet number
Thrombopoietin and the thrombopoietin receptor c-Mpl Normal platelet production
Immune causes of thrombocytopenia
Immune thrombocytopenia Clinical features of primary ITP Pathophysiology of primary ITP Diagnosis of primary ITP Management of children with primary ITP Management of adults with primary ITP Persistent primary ITP after splenectomy Emergency treatment of ITP Drug-induced thrombocytopenia Mechanism of DITP Diagnosis of DITP Heparin-induced thrombocytopenia Clinical features HIT testing Treatment of HIT
Nonimmune causes of thrombocytopenia
Thrombotic microangiopathies Clinical features Pathogenesis Diagnosis Management Splenic sequestration Familial thrombocytopenia Other thrombocytopenic disorders Thrombocytopenia with infection Hemophagocytic syndrome Thrombocytopenia in the critically ill
Disorders of platelet function
Inherited disorders of platelet function Disorders of platelet adhesion Bernard-Soulier Syndrome Von Willebrand disease Disorders of platelet aggregation Glanzmann thrombasthenia Disorders of platelet secretion and signal transduction Deficiency of granule stores Defects in platelet signal transduction and platelet activation Disorders of platelet procoagulant activities Other abnormalities Therapy of inherited platelet function defects Acquired disorders of platelet function Myeloproliferative disorders Acute leukemias and myelodysplastic syndromes Dysproteinemias Uremia Acquired SPD Antiplatelet antibodies and platelet function Drugs that inhibit platelet function   CiteULike  Connotea  Delicious  Digg  Facebook  Reddit  Technorati  Twitter What's this?
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