Day #110 - TTP

Diagnosis:

• Microangiopathic haemolytic anemia (>=5% fragments) with normal INR/aPTT
  
  • Classic pentad:
    
    • Fever
    • Altered mental status
    • Renal failure
    • Microangiopathic haemolytic anemia
    • Thrombocytopenia
  • Few people present with the classic pentad unless left untreated for a long time. Most present with MAHA thrombocytopenia alone.
    
  • Needs to be contrasted with Haemolytic Uremic Syndrome in children with diarrhea (E. coli 0157:H7)
  • Need to exclude DIC from sepsis or malignancy, malignant hypertension, scleroderma renal crisis, HELLP syndrome
    

Causes:

• HIV
  
• Drugs (ticlodipine, clopidogrel, quinine, valacyclovir, cyclosporin A, tacrolimus, others)
• Idiopathic/genetic
• Pregnancy associated

Management:

• Arrange transfer to specialized centre for PLEX
• In interim add steroids for idiopathic TTP
  
• In interim add FFP infusions (i.e. 1u q2h)
  

I have previously blogged about TTP here and anemia (including haemolytic anemia) and thrombocytopenia.