Thrombotic Throbocytopenic Purpura

 

 

Causes and Risk Factors

 

Deficiency of metalloproteinase, responsible for breaking down vWF multimers

 

 

 

 

 

Signs, Symptoms, and Diagnosis

 

  • history
  • physical exam
  • lab investigations
  • diagnostic imaging

History

 

Neurological symptoms

  • headache
  • confusion
  • focal deficits
  • seizures

Physical Exam

 

Fever

 

Lab Investigations

 

Renal failure: abnormal urinalyis, oliguria, ARF

CBC and

  • PT and PTT normal
  • hemolysis: inc uncong bili, LDH, dec haptoglobin
  • negative Coombs' test
  • BUN, creatinine

 

peripheral smear

  • schistocytes and dec platelets

Diagnostic Imaging

 

 

 

 

 

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Pathophysiology

 

vWF is secreted by endothelial cells in a large polymer and is rapidly cleaved by ADAMTS-13.

 

 

 

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Treatments

 

Plasmapheresis and steroids are the treatments of choice. Plasma infusion should be given if plasmapheresis is not immediately available.

Do NOT give platelets, as this can increase rates of intravascular thrombosis.

 

 

 

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Consequences and Course

 

TTP rates of mortality are 90% if untreated.

 

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Resources and References