Thrombotic thrombocytopenic purpura: The role of ADAMTS13

Heesun J Rogers; Charles Allen; Alan E Lichtin

doi:10.3949/ccjm.83a.15009

Thrombotic thrombocytopenic purpura: The role of ADAMTS13

Cleve Clin J Med. 2016 Aug;83(8):597-603. doi: 10.3949/ccjm.83a.15009.

Authors

Heesun J Rogers^{1

2}, Charles Allen³, Alan E Lichtin^{4

5}

Affiliations

¹ Medical Director of Hemostasis and Thrombosis, Hematopathologist, Department of Laboratory Medicine, Cleveland Clinic, Cleveland, OH, USA. E-mail: rogersj5@ccf.org.
² Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA.
³ Department of Laboratory Medicine, Cleveland Clinic, Cleveland, OH, USA.
⁴ Department of Hematologic Oncology and Blood Disorders, Cleveland Clinic; Cleveland, OH, USA.
⁵ Associate Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA.

PMID: 27505881
DOI: 10.3949/ccjm.83a.15009

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an uncommon, life-threatening disease requiring prompt diagnosis and initiation of therapeutic plasma exchange to improve patient survival. However, diagnosis is often difficult because of atypical presentations and signs and symptoms that resemble other conditions. Measurements of ADAMTS13 activity, ADAMTS13 inhibitor, and ADAMTS13 autoantibody are useful for diagnosing TTP, guiding therapy, and predicting relapse.

Publication types

Review

MeSH terms

ADAMTS13 Protein / antagonists & inhibitors
ADAMTS13 Protein / blood*
ADAMTS13 Protein / immunology
Autoantibodies / blood
Humans
Plasma Exchange
Purpura, Thrombotic Thrombocytopenic / blood*
Purpura, Thrombotic Thrombocytopenic / diagnosis
Purpura, Thrombotic Thrombocytopenic / immunology
Purpura, Thrombotic Thrombocytopenic / therapy
Recurrence

Substances

Autoantibodies
ADAMTS13 Protein
ADAMTS13 protein, human