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Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange.
Semin Dial. 2012 Mar-Apr; 25(2):214-9.SD

Abstract

The classification of thrombotic microangiopathy has evolved and expanded due to treatment and mechanistic advances. The two basic clinical forms of thrombotic microangiopathy (excluding disseminated intravascular coagulation [DIC]), thrombotic thrombocytopenic purpura (TTP), and hemolytic uremic syndrome (HUS) encompass a wide range of primary and secondary forms. The advent of plasma therapy and the identification of an inhibitor to ADAMTS13 in the idiopathic or acute forms of TTP and its absence in diarrheal HUS have had a major impact on our current classification of thrombotic microangiopathy. In adults, the difficulty of differentiating TTP, which is much more common than HUS and the need for a speedy diagnosis to provide life-saving plasma therapy has resulted in the term TTP/HUS for adult forms of thrombotic microangiopathy that present with unexplained thrombocytopenia and microangiopathic hemolytic anemia without a DIC. In this adult population a primary idiopathic and hereditary form as well as eight known secondary categories or clinical forms of TTP/HUS have been identified. HUS also embraces a primary (atypical HUS) and secondary forms (majority, diarrheal HUS secondary to Escherichia coli 0157:H7). In children, who present with HUS with no preceding history of diarrhea, plasma therapy is also offered on an urgent basis and studies are carried out to determine if they are suffering an abnormality in complement activation that may require eculizumab therapy. The advent of plasma therapy in the treatment of thrombotic microangiopathy has led to a clearer understanding of the role of ADAMTS13, both short- and long-term outcomes and the need for future surveillance and intervention.

Authors+Show Affiliations

Department of Medicine, University of Western Ontario, London, Ontario, Canada. william.clark@lhsc.on.ca

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

22309967

Citation

Clark, William F.. "Thrombotic Microangiopathy: Current Knowledge and Outcomes With Plasma Exchange." Seminars in Dialysis, vol. 25, no. 2, 2012, pp. 214-9.
Clark WF. Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange. Semin Dial. 2012;25(2):214-9.
Clark, W. F. (2012). Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange. Seminars in Dialysis, 25(2), 214-9. https://doi.org/10.1111/j.1525-139X.2011.01035.x
Clark WF. Thrombotic Microangiopathy: Current Knowledge and Outcomes With Plasma Exchange. Semin Dial. 2012 Mar-Apr;25(2):214-9. PubMed PMID: 22309967.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange. A1 - Clark,William F, Y1 - 2012/02/07/ PY - 2012/2/8/entrez PY - 2012/2/9/pubmed PY - 2012/8/1/medline SP - 214 EP - 9 JF - Seminars in dialysis JO - Semin Dial VL - 25 IS - 2 N2 - The classification of thrombotic microangiopathy has evolved and expanded due to treatment and mechanistic advances. The two basic clinical forms of thrombotic microangiopathy (excluding disseminated intravascular coagulation [DIC]), thrombotic thrombocytopenic purpura (TTP), and hemolytic uremic syndrome (HUS) encompass a wide range of primary and secondary forms. The advent of plasma therapy and the identification of an inhibitor to ADAMTS13 in the idiopathic or acute forms of TTP and its absence in diarrheal HUS have had a major impact on our current classification of thrombotic microangiopathy. In adults, the difficulty of differentiating TTP, which is much more common than HUS and the need for a speedy diagnosis to provide life-saving plasma therapy has resulted in the term TTP/HUS for adult forms of thrombotic microangiopathy that present with unexplained thrombocytopenia and microangiopathic hemolytic anemia without a DIC. In this adult population a primary idiopathic and hereditary form as well as eight known secondary categories or clinical forms of TTP/HUS have been identified. HUS also embraces a primary (atypical HUS) and secondary forms (majority, diarrheal HUS secondary to Escherichia coli 0157:H7). In children, who present with HUS with no preceding history of diarrhea, plasma therapy is also offered on an urgent basis and studies are carried out to determine if they are suffering an abnormality in complement activation that may require eculizumab therapy. The advent of plasma therapy in the treatment of thrombotic microangiopathy has led to a clearer understanding of the role of ADAMTS13, both short- and long-term outcomes and the need for future surveillance and intervention. SN - 1525-139X UR - https://www.unboundmedicine.com/medline/citation/22309967/Thrombotic_microangiopathy:_current_knowledge_and_outcomes_with_plasma_exchange_ L2 - https://doi.org/10.1111/j.1525-139X.2011.01035.x DB - PRIME DP - Unbound Medicine ER -