hemostasis

• regulatory mechanism of the coagulation cascade

  Fibrinolysis
• becomes bound to fibrin at the time of fibrin polymerization


  Plasminogen
• main enzyme of the fibrinolytic system

  plasmin
•  serine proteases that systematically digest fibrin

  plasmin
• also digest factor V, VIII and fibrinogen

  Plasmin
• characteristics of Immune Thrombocytopenic purpura:

  -destruction of platelets

  -impaired platelet production

  -IgG directed to GPIIb/IIIa, GP1b/IX, GPV
• #1: children

  
• #2: <20 x 109/L

  
• #3: Abrupt

  
• #4: common

  
• #5: common

  
• #6: corticosteroids, anti-D, IV-IG

  
• #7: Adult

  
• #8: <30-80 x 109/L

  
• #9: Insidious

  
• #10: unusual

  
• #11: rare

  
• #12: corticosteroids, splenectomy

  
• thrombocytopenic purpura characteristics (3):

  -deficiency in ADAMTS13

  -presence of large VWF multimers

  -congenital: upshaw-schulman syndrome
• Hemolytic Uremic Syndrome characteristics(2):

  -deficiency in proteins that regulates the alternative pathway

  -adult onset: bacterial infections, connective tissue disorders and some type of cancer.
• occurs in Adults: 20-50 years old


  Thrombotic thrombocytopenic purpura
• occurs in children <5 years old


  Hemolytic Uremic syndrome
• type of microangiopathic hemolytic anemia that classically has been characterized by the pentad of fever, thrombocytopenia, hemolytic anemia, renal dysfunction, and neurologic dysfunction.

  Thrombotic thrombocytopenic purpura
• characterized by the triad of thrombotic microangiopathy, thrombocytopenia, and acute kidney injury

  Hemolytic Uremic Syndrome