The Clinical Spectrum of Thrombotic Thrombocytopenic Purpura

Hau C. Kwaan; Lisa N. Boggio

doi:10.1055/s-2005-925473

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2005; 31(6): 673-680
DOI: 10.1055/s-2005-925473

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

Abstract

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

Full Text

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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