复旦大学附属中山医院：特发性血小板减少性紫癜 DISORDERS OF HEMOSTASIS

DISORDERS OF HEMOSTASIS Dept of Hematology ZhongShan Hospital, FuDan University Prof, JIAn-mInXU

DISORDERS OF HEMOSTASIS Dept.of Hematology ZhongShan Hospital,FuDan University Prof. JlAN-MIN XU

General Considerations Disorders of hemostasis defects in platelet number or function problems in fibrin clot (coagulation Bleeding due to platelet disorders is typically mucosal or dermatologic include epistaxis, gum bleeding menorrhagia, gastrointestinal bleeding purpura, and petechiae

General Considerations • Disorders of hemostasis defects in platelet number or function problems in fibrin clot (coagulation) • Bleeding due to platelet disorders is typically mucosal or dermatologic include epistaxis, gum bleeding, menorrhagia, gastrointestinal bleeding, purpura, and petechiae

Thrombocytopenic Purpura Dept of Hematology ZhongShan Hospital, FuDan University Prof, JIAn-mInXU

Thrombocytopenic Purpura Dept.of Hematology ZhongShan Hospital,FuDan University Prof. JlAN-MIN XU

Brief 1.definition bleeding of skin, mucous and organs 2. cause: (1)production :I drug, tumor, infection, adiation (2)destruction :t ITP, DIC, TTP (3)abnormal distribution: splenomegaly 3.classification primary secondary

Brief 1.definition：bleeding of skin、mucous and organs 2.cause： （1）production ：drug, tumor, infection, radiation （2）destruction ：ITP,DIC,TTP （3）abnormal distribution：splenomegaly 3.classification：primary ,secondary

IDIOPATHIC (AUTOIMMUNE THROMBOCYTOPENIC PURPURA(TP)

IDIOPATHIC (AUTOIMMUNE) THROMBOCYTOPENIC PURPURA (ITP)

General Considerations ITP is an autoimmune disorder in which an lag autoantibody is formed that binds to platelets It is not clear which antigen on the platelet surface is involved. Although the antiplatelet antibody may bind complement, platelets are not destroyed by direct lysis

General Considerations • ITP is an autoimmune disorder in which an IgG autoantibody is formed that binds to platelets • It is not clear which antigen on the platelet surface is involved. • Although the antiplatelet antibody may bind complement, platelets are not destroyed by direct lysis

General Considerations Rather, destruction takes place in the spleen, where splenic macrophages with Fc receptors bind to antibody coated platelets Since the spleen is the major site both of antibody production and platelet sequestration, splenectomy is highly effective therapy

General Considerations • Rather, destruction takes place in the spleen, where splenic macrophages with Fc receptors bind to antibody￾coated platelets • Since the spleen is the major site both of antibody production and platelet sequestration, splenectomy is highly effective therapy

General Considerations 1.definition: thrombocyte unrelated to a drug, an infection, or autoimmune disease is generally classified as ITP 2 character: shortened span of platelet increased megakaryocyte in bone marrow 3 classification: acute, chronic

General Considerations 1.definition：thrombocyte unrelated to a drug, an infection, or autoimmune disease is generally classified as ITP 2.character： shortened span of platelet increased megakaryocyte in bone marrow 3.classification：acute, chronic

Mechanism 1.Immunity (1 acute postviral infection immune complex(viral Ag and Ab) connect with platelet Fc-R viral components banding with platelet auto Ab generation (2) chronic: not affected by foreign Ag

Mechanism 1.Immunity （1）acute： postviral infection immune complex (viral Ag and Ab) connect with platelet Fc-R viral components banding with platelet auto Ab generation （2）chronic：not affected by foreign Ag

Mechanism 2. iver and spleen: (1)1/3 platelets are held within the spleen (2) spleen produces platelet associated Ab (3) liver and spleen erase platelet 7-11 days in normal persons, 1-3 days in patients

Mechanism 2.liver and spleen： （1）1/3 platelets are held within the spleen （2）spleen produces platelet associated Ab （3）liver and spleen erase platelet：7-11 days in normal persons, 1-3 days in patients