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Semin Thromb Hemost 2005; 31(6): 673-680
DOI: 10.1055/s-2005-925473
Copyright © 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

The Clinical Spectrum of Thrombotic Thrombocytopenic Purpura

Hau C. Kwaan1 , 2 , Lisa N. Boggio2
  • 1Professor of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois
  • 2Division of Hematology/Oncology, Northwestern University Feinberg School of Medicine, Chicago, Illinois
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Publication History

Publication Date:
30 December 2005 (online)

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ABSTRACT

The clinical syndrome of fever, neurologic abnormalities, renal impairment with laboratory findings of thrombocytopenic and microangiopathic hemolytic anemia is seen in thrombotic thrombocytopenic purpura (TTP) and a variety of disorders associated with thrombotic microangiopathy (TMA). With improved understanding of the pathogenesis of the perturbed metabolic pathway of von Willebrand factor in TTP, the classic Moschcowitz syndrome, now more accurately referred to as idiopathic TTP, can be distinguished from other TMAs. The distinguishing features are useful not only in providing an accurate diagnosis but also help to determine the best therapeutic strategy.

KEYWORDS

Thrombotic thrombocytopenic purpura - thrombotic microangiopathy - hemolytic uremic syndrome - ADAMTS13 - plasma exchange

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Hau C KwaanM.D. 

Professor, Division of Hematology/Oncology, Northwestern University Medical School, VA Lakeside Medical Center

333 East Huron Street, Chicago, IL 60611

Email: h-kwaan@northwestern.edu