w血小板减少性紫癜课件.ppt

Thrombocytopenic purpura Huang Honghui Dept. of Hematology, Renji Hospital Definition: Thrombocytopenia is a clinical syndrome in which a decreased number of platelets in the circulating blood present as a bleeding tendency, i.e. skin, mucosa or internal organ bleeding. It is the most common course of abnormal bleeding(30%). Classification: idiopathic/ secondary BPC50000/μl----bleeding tendency BPC20000/μl----spontaneous hemorrhage Idiopathic Thrombocytopenia Purpura(ITP) Definition ITP is an acquired disease of children and adults characterized by a low platelet count, an normal or increased numbers of megakaryocytes in the bone marrow, and absence of evidence for other disease. Classification Acute type (aITP) Chronic type (cITP) Etiology and Pathogenesis Immunologic processes The infusion of plasma from patients with ITP into normal recipients → thrombocytopenia The infusion of normal platelets into patients with ITP → destructed within 12-24h platelet associated antibodies ( PAIgG, PAIgA, PAIgM ) Glucocorticoid, plasmapheresis, HD-Ig --- good response In 1951 Dr William Harrington infused himself with plasma from an ITP patient and developed severe but transient thrombocytopenia Since then platelet destruction in ITP is believed to be mediated by platelet autoantibodies Etiology and Pathogenesis Role of the liver and spleen The site of production of platelet antibodies. Etiology and Pathogenesis Role of the liver and spleen The site of platelet clearance. Splenic sequestration is the major site of platelet clearance in ITP.( 51Cr-labeled isologous platelet ) Hepatic sequestration ---- severe thrombocytopenia and markedly shorten platelet survival. Etiology and Pathogenesis 51Cr labeled isologous platelet Administrate to patients with ITP External scintillation counting Rapid accumulation of radioactivity predominantly in the spleen Splenic sequestration is the major site of platelet clearance in ITP Etiology and Pathogenesis