• Classic findings in TTP and other primary thrombotic microangiopathies
  • Fever
  • Anemia
  • Thrombocytopenia
  • Renal failure
  • Altered sensorium
  
  On blood smear, see both schistocytes (3-5 per field) and polychromasia due to reticulocytosis. 
  
  

  Posted 12/06/16 11:36:05 AM by Anna Krigel

• Primary TMA Syndromes
  • TTP - hereditary or acquired, TMA resulting from ADAMTS13 deficiency (defined as activity <10%)
    • Upshaw-Shulman syndrome = hereditary deficiency of ADAMTS13
    • Acquired TTP results from inhibition of ADAMTS13 activity by an autoantibody
  • Shiga toxin-mediated HUS
  • Drug-induced TMA syndromes
  • Complement-mediated TMA - hereditary or acquired
  
  

  Posted 12/06/16 04:52:04 PM by Anna Krigel

• Management of TMA syndromes
  • Plasma exchange - started urgently in all patients with suspected TTP and no obvious underlying cause for another TMA syndrome; it is not recommended to wait for ADAMTS13 activity based on high mortality of TTP without plasma exchange; unclear if plasma exchange helps in typical HUS
  • Glucocorticoids - also given in acquired TTP for immunosuppression
  • Eculizumab - monoclonal Ab to complement factor C5, used in patients with complement-mediated HUS, has been shown in case series to be beneficial in patients with typical HUS and neurologic symptoms
  
  

  Posted 12/06/16 06:44:18 PM by Anna Krigel

• For further reading...

  Plasma Exchange for TTP (NEJM 1991)
  Eculizumab in Atypical HUS
  
  

  Posted 12/06/16 06:49:27 PM by Anna Krigel

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