Back to Basics: Thrombotic Thrombocytopenic Purpura (TTP)

Back to Basics: Thrombotic Thrombocytopenic Purpura (TTP)

Classic Pentad (rarely all present):

  • Fever
  • Microangiopathic hemolytic anemia
  • Thrombocytopenia
  • Renal pathology
  • CNS abnormalities

Risk Factors:  pregnancy, infection (HIV, pancreatitis, sepsis, etc.), malignancy, surgery, drugs (ciprofloxacin, ofloxacin, levofloxacin, quinine, clopidogrel, ticlopidine, etc.)

 

Pathophys: decreased activity of ADAMTS-13 which normally functions to cleave von Willebrand factor

 

Labs:  anemia, thrombocytopenia, increased LDH, decreased haptoglobin, increased total bili, normal PT/PTT/INR (differs from DIC), hemoglobinuria

 

Treatment: plasma exchange therapy, if plasmapheresis cannot be performed immediately then fresh frozen plasma infusion (contains ADAMTS-13) should be initiated, avoid platelet transfusions because can acutely worsen thrombosis leading to renal failure and possible death

 

References:

George, J. “Thrombotic thrombocytopenic purpura.”  New England Journal of Medicine. 2006; 354:1927.

Tintinalli, JE. Tintinalli's Emergency Medicine: A Comprehensive Study Guide. 8th ed.  New York, NY: McGraw-Hill Education LLC, 2016.