Premium
Atypical presentations of thrombotic thrombocytopenic purpura: A review
Author(s) -
Sarode Ravi
Publication year - 2008
Publication title -
journal of clinical apheresis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.697
H-Index - 46
eISSN - 1098-1101
pISSN - 0733-2459
DOI - 10.1002/jca.20182
Subject(s) - thrombotic thrombocytopenic purpura , medicine , microangiopathic hemolytic anemia , adamts13 , von willebrand factor , therapeutic plasma exchange , plasmapheresis , intensive care medicine , schistocyte , presentation (obstetrics) , pathophysiology , pediatrics , platelet , immunology , surgery , antibody
Thrombotic thrombocytopenic purpura (TTP) is diagnosed by the presence of microangiopathic hemolytic anemia and thrombocytopenia in a patient who frequently presents with central nervous system involvement and, to a lesser extent, renal dysfunction. Recent understanding of the pathophysiology of TTP due to severe deficiency of von Willebrand factor cleaving protease, known as ADAMTS13, has improved diagnosis of TTP. Once the diagnosis is suspected, life‐saving therapeutic plasma exchange therapy is initiated. Occasionally, an unusual clinical presentation makes TTP diagnosis difficult, thus resulting in a delay in the management of TTP. This review highlights a variety of atypical TTP presentations described in the literature. It is intended to bring unusual scenarios to the clinician's awareness, so that timely treatment can be delivered. J. Clin. Apheresis, 2009. © 2008 Wiley‐Liss, Inc.