What's new in the diagnosis and pathophysiology of thrombotic thrombocytopenic purpura

Severe ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) deficiency causes thrombotic thrombocytopenic purpura (TTP), which is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and the absence of oliguric or anuric renal failure. However...

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Bibliographic Details
Published in:Hematology 2015-12, Vol.2015 (1), p.631-636
Main Author: Sadler, J Evan
Format: Article
Language:English
Subjects:
ADAM Proteins - blood
ADAM Proteins - deficiency
ADAMTS13 Protein
Arteries - pathology
Hematology - methods
Hematology - trends
Hemolysis
Humans
Nervous System Diseases - complications
Purpura, Thrombotic Thrombocytopenic - diagnosis
Purpura, Thrombotic Thrombocytopenic - physiopathology
Renal Insufficiency - complications
Thrombosis - complications
Venous Thrombosis - complications
von Willebrand Factor - metabolism
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Summary:Severe ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) deficiency causes thrombotic thrombocytopenic purpura (TTP), which is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and the absence of oliguric or anuric renal failure. However, some patients with this constellation of findings do not have ADAMTS13 deficiency, and some patients with ADAMTS13 deficiency have renal failure or relatively normal blood counts. Consequently, many investigators and clinicians have incorporated severe ADAMTS13 deficiency into the case definition of TTP. This change has facilitated the timely initiation of treatment for patients with atypical clinical features who otherwise would not be recognized as having TTP. Conversely, excluding severe ADAMTS13 deficiency focuses attention on the diagnosis and treatment of other causes of thrombotic microangiopathy that require different treatment. The rapid return of ADAMTS13 data is important to make the best use of this information.
ISSN:1520-4391
1520-4383
DOI:10.1182/asheducation-2015.1.631