Biochemistry of Coagulation - Goueli Flashcards
What is Hemostasis?
Physiological blood clotting in response to injury or vascular leak (keeping blood where it belongs)
What is Thrombosis?
Pathologic blood clotting
What is Hemorrhage?
Pathologic bleeding
What is involved in Hemostasis?
The process of coagulation and subsequent dissolution of clot
What is the process of clot degradation?
Fibrinolysis
What is a Primary Hemostasis?
Platelets become activated and aggregate at the site of injury, forming a temporary, loose platelet plug
What is a normal platelet count?
150,000 - 450,000 platelets
What are patients at risk for with
Increased risk of spontaneous hemorrhage
What are patients more at risk for with risk of spontaneous hemorrhage?
Intracranial hemorrhage
What are patients at risk for with
Increased difficulty clotting if already bleeding
What is the pathogenic issue in vonWillebrand’s disease?
Complex multimeric glycoprotein produced by and stored in the alpha granules of platelets; synthesized by megakaryocytes and found in subendothelial connective tissue
What is Bernard Soulier Syndrome?
Glycoprotein 1B deficiency
What is TTP (Thrombotic Thrombocytopenic Purpura)?
Characterized by profound thrombocytopenia and microangiopathic hemolytic anemia (red blood cell fragmentation)
What is the major cause of TTP?
Deficiency in ADAMTS13
What is ADAMTS13?
A disintegrin and metalloproteinase with thrombospondin Type 1 motif 13
What happens with ADAMTS13 is absent?
Persistence of conglomerates which causes platelet aggregation when the protein is subjected to high shear stress (due to lack of cleavage of high molecular weight vonWillebrand’s protein)
What are the 5 clinical features of TTP?
Thrombocytopenia Red Cell Fragmentation Fever Transient Neurologic Deficits Kidney Failure
What is the treatment of TTP?
Plasmaphoresis