L3 Hemostasis II Flashcards
Disorders of primary hemostasis
Mucocutaneous bleeding
Prolonged bleeding
Decreased platelet count
Disorders of secondary hemostasis
soft tissue bleeding
Prolonged
Prothrombin T, Partial Thromboplatstin T, Thrombin time
Factor VIII Complex
Components
vWF (platelets and endothelial cells) Factor VIII (Liver)
Von Willebrand disease
Type of disease
I, II, III
Autosomal dominant
I:Decreased vWF
II: Abnormal vWF
III: No vWF
von Willebrand disease
Treatment
Mild: desmopressin (releases vWF from endothelial)
Severe: VIII w/ vWF and cryoprecipitate
Hemophilia A
Type of disorder
What is it
X linked recessive
Decreased VIII production
Soft tissue bleeding
Hemophilia A Lab findings
Normal bleeding time Prolonged PTT Decreased VIII normal vWF Normal IX
Hemophilia B
Type of disorder
What is it
X linked recessive
Decreased IX production
Thrombocytopenia
Count
Symptoms
<100,000/uL
Spontaneous bleeding at 20,000
Petechial hemorrhages in skin and mucous membranes
Immune Thrombyctopenic Purpura
Acute vs chronic
Immne mediated distruction of platelets
Acute: children, severe,2-6 weeks, boys and girls
Chronic: adults, mild, females
Immune thrombocytopenic purpura treatment
Corticosteroids
IV immunoglobulin
Splenectomy
Thrombotic Thrombocytopenic Purpura
What is it
Intravascular platelet activation w/
Formation of platelet rich microthrombi throughout circulation
Accumulation of large vWF
Thrombotic Thrombocytopenic Purpura
Cause
Deficiency of metalloproteinase ADAMTS 13
usually degrades high MW vWF
Disseminated intravascular coagulation
Cause
uncontrolled activation of hemostatic system
bleeding and microvascular thrombi