文档介绍：REGULATION OF COAGULATION/DISSEMINATED INTRAVASCULAR COAGULATION
REGULATION OF COAGULATION Introduction
Coagulation necessary for maintenance of vascular integrity
Enough fibrinogen to clot all vessels
What controls clotting process?
COAGULATION CASCADE
Va/Xa/PL
VIIIa/IXa/PL
or
VIIa/TF
FXII
FXIIa
TF
FVII
FG
F
HMWK
FXI
FXIa
VIIa/TF
or
FVIIa
Ca+2
FIX
FIXa
Ca+2
Ca+2
Ca+2
VIII
VIIIa
T
V
Va
T
Ca+2
FX
FXa
Ca+2
Ca+2
PT
T
Common Pathway
ponents
Surface Active Components
INTRINSIC PATHWAY
EXTRINSIC PATHWAY
COAGULATION INHIBITORS
Tissue Factor Pathway Inhibitor (TFPI)
Lipoprotein Associated Coagulation Inhibitor (LACI) Extrinsic Pathway Inhibitor (EPI)
Complexes with Factors VIIa/TF/Xa; inactivates Xa
Antithrombin III/Heparin Cofactor II/Heparin
Binds and Inactivates Enzymes
Protein C/Protein S/Thrombomodulin
Cleaves & Inactivates Cofactors (Va & VIIIa)
Plasminogen - 3º hemostasis
Cleaves Fibrin
ATIII
+
Thrombin
Heparin
ANTITHROMBIN III - MECHANISM OF ACTION
PROTEIN C ACTIVATION
Protein C
+
Thrombin
Activated
Protein C
T
T
T
M
Protein C
Activated
Protein C
Thrombin
Thrombomodulin
+
*
*
Ca
PROTEIN C - MECHANISM OF ACTION
APC
Factor Va/Factor VIIIa
Pro S PL
iFVa/FVIIIa
ANTICOAGULANT PROTEIN DEFICIENCY Disease entities
Heterozygous Protein Deficiency
Increased Venous Thrombosis
Occasional Increased Arterial Thrombosis
Warfarin Induced Skin Necrosis
Homozygous Protein Deficiency
Neonatal Purpura Fulminans
Fibrinogenolysis
Chronic DIC
ANTICOAGULANT PROTEIN DEFICIENCY
Dominant
Increased Venous Thrombosis
Young Age of Thrombosis
No Predisposing Factors to Thrombosis
Increased Thrombin Generation
Positive Family History
Recessive
No history of thrombosis
No family history
Neonatal Purpura Fulminans
Increased Thrombin Generation