Thrombotic Microangiopathy

  • Piero Ruggenenti
  • Giuseppe Remuzzi

Abstract

The term thrombotic microangiopathy (TMA) defines a lesion of vessel wall thickening (mainly arteriole or capillaries) with swelling and/or detachment of the endothelial cell from the basement membrane and accumulation of fluffy material in the subendothelial space. Both widening of the subendothelial space and intraluminal platelet thrombi lead to partial or complete obstruction of the vessel lumina. Erythrocytes forced into the narrowed vessels are disrupted by a mechanical trauma, and fragmented and distorted erythrocytes can thus be observed in the circulation. Fragmented erythrocytes are pathognomonic for the microangiopathic hemolytic anemia associated with thrombotic microangiopathy. The term thrombotic microangiopathy was first introduced in 1952 by William St. Clair Symmers to replace thrombotic thrombocytopenic purpura (TIP) and has been employed in more recent time by nephrologists to indicate renal lesions of the hemolytic uremic syndrome (HUS) (1). It derives from the fact that the vascular lesions of HUS and TTP are virtually indistinguishable, a point recognized by Habib and coworkers in 1958 (2). Very similar lesions are found in scleroderma and malignant hypertension (3). Within the limits of the assumption that HUS and TTP are different clinical expression of the same disease—with renal failure predominating in young children and brain involvement in adults—the term thrombotic microangiopathy, as revived in 1979 by Ennio C. Rossi (4), is still the best term to encompass all these syndromes.

Keywords

Plasma Exchange Hemolytic Uremic Syndrome Fresh Freeze Plasma Thrombotic Thrombocytopenic Purpura Thrombotic Microangiopathy 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1998

Authors and Affiliations

  • Piero Ruggenenti
    • 1
  • Giuseppe Remuzzi
    • 1
  1. 1.Mario Negri Institute for Pharmacological Research, Division of Nephrology and DialysisOspedali Riuniti de BergamoBergamoItaly

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