Thrombotic thrombocytopenic purpura

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Thrombotic thrombocytopenic purpura is a type of thrombotic microangiopathy.

Classic pentad

It was classically described as the pentad of:[1]

  1. Fever.
  2. Thrombocytopenia.
  3. Microangiopathic hemolytic anemia.
    • Usu. with schistocytes (fragmented RBCs).[2]
  4. Transient neurologic symptoms.
  5. Renal failure.

Mnemonic

HUS/TTP is a CRAFTY syndrome:[3]

  • CNS symptoms.
  • Renal failure.
  • Anemia - microangiopathic hemolytic anemia.
  • Fever.
  • Thrombocytopenia.
  • Y - no one knows "Y" it occurs.

Etiology

Deficiency of plasma enzyme ADAMTS13 which may be:[1]

  • Inherited.
  • Acquired (autoantibodies).

See also

References

  1. 1.0 1.1 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 669. ISBN 978-1416031215.
  2. Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 65. ISBN 978-1416002741.
  3. URL: http://www.valuemd.com/mnemonics2.php. Accessed on: 8 November 2010.