Coagulation Cascade - Cloting Factors & CC
Terms
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- Factor XII
- Hageman Factor - binds to exposed collagen; Activated by HMWK and PK
- Factor XI
- PTA - activated by factor XIIa
- Factor IX
- Christmas Factor, PTC - activated by factor XIa in presence of Ca2+
- Factor VII
- Pronvertin SPCA - Activated by Thrombin in the presence of Ca2+; in tissue
- Factor X
- Stuart-Prower Factor - Activates on surface of platelets, VIIa, TF, Ca2+
- Factor II
- Prothrombin - Activated on surface of platelets, prothrombinase complex
- Factor VIII
- Antihemophilia factor a, AHG - activated by thrombin; factor VIIIa is cofactor for X by IX activation
- Factor III (TF)
- Tissue Factor - sub endothelial cell surface glycoprotein; cofactor for VII
- Factor I
- Fibrinogen - cleaved by thrombin to form fibrin clot
- Factor XIII
- Fibrin Stabilizer - Activated by thrombin in presence of Ca2+
- von Willebrand Factor
- associated with sub endothelial connective tissue; serves as bridge between platelets glycoprotein GbIb/IX and collagen
- Protein C
- Activated to Ca by thrombin bound to thrombomodulin; degrades factors VIIIa and Va
- Protein S
- Cofactor for protein C; both contain gla residues
- Thrombomodulin
- Protein on surface of endothelial cells; binds thrombin to activate protein C
- Antithrombin III
- Most important coagulation inhibitor; controls activities of IIa, factors IXa, Xa, XIa and XIIa
- Prekallikrein - PK
- Fletcher Factor
- High Molecular Weigh Kininogen - HMWK
- Contact activation factor
- Factor IV
- CALCIUM yo!!!
- Hemophilia A
-
-Factor VIII
-X-linked w/ severe, mod, mild
-150 mutations
-1:5000, 1:10,000 males
-joint and muscle hemorrhage
-easy bruising & prolonged bleeding
-treatment w/ VIII concentrates - Hemophilia B
-
-Factor IX
-1/10 of A prevelance
-X-linked w/ severe, mod, mild
-300 mutations
-85% point mutations
-3% short nucleotide deletions or insertions
-12% gross gene alterations - Disorders of Fibrinogen & Factor XIII
-
-elevated levels in patients with CHD, hperlipidemias, diabetes and hypertension
-in pregnancy, menopause, women on oral contraceptives and smokers
-other rare disorders show complete lack or deficiency
-Afibringenemia: neonatal umbilical cord hemorrhage, mucosal hemorrhagen and recurrent abortion
-Factor XIII - rare - von Willebrand Disease
-
-most common
-due to inherited deficiency in vWF
-most commonly inherited bleeding disorder
-defective platelet adhesion (VIII)
-heterogeneous disorder type 1, 2, and 3
-Autosomal dominant - Factor XI and Contact Adhesion
-
-deficiency of Factor XI results in injury-related bleeding
-common in Ashkenazic jews
-autosomal dominantL: three independent point mutations - Antithrombin Deficiency
-
-Deficiency ranges from 1:2000 to 1:5000 in general pop
-deficiency arise from mutations that affect synthesis or stability and from mutations that affect protease and/or heparin binding sites
-clinical consequences are deep vein thrombosis and pulmonary embolism
-acute episodes and treated with heparin infusion