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What is hemostasis?
The process that stops bleeding while maintaining blood in a fluid state within blood vessels
What are the two major disorders of hemostasis?
Hemorrhage (excessive bleeding) and thrombosis (abnormal clot formation)
What is thrombosis?
Abnormal formation of a blood clot within a blood vessel
What is hemorrhage/purpura?
Excessive bleeding due to defects in hemostasis
What is the normal platelet reference range?
150,000–400,000/µL
What is a quantitative platelet disorder?
Abnormal platelet number
What is a qualitative platelet disorder?
Abnormal platelet function
Which platelet disorder is due to defective aggregation?
Glanzmann thrombasthenia
What are the three components of hemostasis?
Extravascular, vascular, and intravascular
What is the role of the extravascular component?
Tissue contraction around the vessel to lessen bleeding
Why does less surrounding muscle cause more bleeding?
Less tissue contraction around the damaged vessel
What structures are included in the vascular component?
Arteries, veins, capillaries, arterioles, and venules
Which connective tissue disorders cause vascular bleeding?
Ehlers-Danlos syndrome and Marfan syndrome
What makes up the intravascular component?
Platelets, coagulation proteins, inhibitors, control proteins, and fibrinolytic proteins
What is primary hemostasis?
Formation of a temporary platelet plug
How fast does primary hemostasis occur?
Seconds to minutes
Which test assesses primary hemostasis?
Bleeding time
Which factors affect bleeding time?
Platelets, blood vessels, vWF, fibrinogen, and calcium
Name two bleeding time methods.
Duke method and Ivy method
What protein allows platelet adhesion to blood vessels?
Von Willebrand factor
What is secondary hemostasis?
Stabilization of the platelet plug with fibrin
Which test assesses secondary hemostasis?
Clotting time
Name two clotting time methods.
Lee-White method and slide/drop method
What is the normal platelet lifespan?
7–10 days
Where are platelets produced?
Bone marrow from megakaryocytes
What percentage of platelets circulate in blood?
70%
What percentage of platelets are sequestered in the spleen?
30%
What condition causes thrombocytopenia due to splenic pooling?
Splenomegaly
What condition causes thrombocytosis?
Splenectomy
Which platelet granules contain vWF and fibrinogen?
Alpha granules
Which platelet granules contain ADP and calcium?
Dense granules
What is the first step of platelet plug formation?
Platelet adhesion
Which platelet receptor is responsible for adhesion?
GPIb
What bridges platelets to exposed collagen?
Von Willebrand factor
Which platelet receptor is responsible for aggregation?
GPIIb/IIIa
What molecule links platelets during aggregation?
Fibrinogen
Which platelet substance amplifies aggregation?
ADP
Which platelet-derived substance causes vasoconstriction and aggregation?
Thromboxane A2
Which drugs inhibit thromboxane A2 formation?
Aspirin and NSAIDs
What is the fastest coagulation pathway?
Extrinsic pathway
Which factors belong to the extrinsic pathway?
Factor III (tissue factor) and Factor VII
Which pathway is activated by collagen exposure?
Intrinsic pathway
Which factors belong to the intrinsic pathway?
Factors XII, XI, IX, VIII, HMWK, and prekallikrein
Which pathway is common to PT and PTT?
Common pathway
Which factors belong to the common pathway?
Factors I, II, V, X, and XIII
Which factor stabilizes fibrin?
Factor XIII
Which clotting factor is calcium?
Factor IV
What does prothrombin time (PT) assess?
Extrinsic and common pathways
What is the normal PT reference range?
12–14 seconds
What does partial thromboplastin time (PTT) assess?
Intrinsic and common pathways
What is the normal PTT reference range?
20–45 seconds
Which factor deficiency prolongs PTT only?
Factor VIII, IX, XI, or XII deficiency
Which factor deficiency prolongs PT only?
Factor VII deficiency
What is fibrinolysis?
Breakdown and removal of the fibrin clot
What is the inactive precursor of plasmin?
Plasminogen
What converts plasminogen to plasmin?
Plasminogen activators
What is the main endogenous plasminogen activator?
Tissue plasminogen activator (t-PA)
What does plasmin break down?
Fibrin and fibrinogen
What laboratory marker indicates fibrin breakdown?
D-dimer
Which hemophilia is caused by Factor VIII deficiency?
Hemophilia A
Which hemophilia is caused by Factor IX deficiency?
Hemophilia B
Which hemophilia is caused by Factor XI deficiency?
Hemophilia C
What autoimmune inhibitor most commonly affects coagulation?
Factor VIII inhibitor
What causes hypercoagulability due to loss of regulation?
Protein C or Protein S deficiency
What does lupus anticoagulant do in vitro versus in vivo?
Inhibits clotting in vitro and promotes thrombosis in vivo
What cell produces platelets?
Megakaryocyte
What process allows nuclear division without cytoplasmic division in megakaryocytes?
Endomitosis
How long does megakaryocyte development take?
5–7 days
What hormone stimulates megakaryocyte maturation?
Thrombopoietin
Which interleukins support megakaryopoiesis?
IL-3, IL-6, IL-7, and IL-11
What is the final stage of megakaryocyte development?
Metamegakaryocyte