Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura

Kidney Int. 2001 Sep;60(3):831-46. doi: 10.1046/j.1523-1755.2001.060003831.x.

Abstract

The term thrombotic microangiopathy (TMA) defines a lesion of vessel wall thickening (mainly arterioles or capillaries), intraluminal platelet thrombosis, and partial or complete obstruction of the vessel lumina. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different entities have been described: the hemolytic uremic syndrome (HUS) and the thrombotic thrombocytopenic purpura (TTP). Injury to the endothelial cell is the central and likely inciting factor in the sequence of events leading to TMA. Loss of physiological thromboresistance, leukocyte adhesion to damaged endothelium, complement consumption, abnormal von Willebrand factor release and fragmentation, and increased vascular shear stress may then sustain and amplify the microangiopathic process. Intrinsic abnormalities of the complement system and of the von Willebrand factor pathway may account for a genetic predisposition to the disease that may play a paramount role in particular in familial and recurrent forms. Outcome is usually good in childhood, Shiga toxin-associated HUS, whereas renal and neurological sequelae are more frequently reported in adult, atypical, and familial forms of HUS and in TTP. Plasma infusion or exchange is the only treatment of proven efficacy. Bilateral nephrectomy and splenectomy may serve as rescue therapies in very selected cases of plasma resistant HUS or recurrent TTP, respectively.

Publication types

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

MeSH terms

  • Adult
  • Antigen-Antibody Complex
  • Capillaries / pathology
  • Child
  • Complement Factor H / deficiency
  • Complement Factor H / genetics
  • Drug-Related Side Effects and Adverse Reactions
  • Endothelium, Vascular / pathology
  • Escherichia coli Infections / complications
  • Guidelines as Topic
  • Hemolytic-Uremic Syndrome / etiology*
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / pathology
  • Hemolytic-Uremic Syndrome / therapy
  • Humans
  • Kidney Glomerulus / blood supply
  • Lipopolysaccharides
  • Purpura, Thrombotic Thrombocytopenic / etiology*
  • Purpura, Thrombotic Thrombocytopenic / genetics
  • Purpura, Thrombotic Thrombocytopenic / pathology
  • Purpura, Thrombotic Thrombocytopenic / therapy
  • Shiga Toxin

Substances

  • Antigen-Antibody Complex
  • Lipopolysaccharides
  • complement factor H, human
  • Shiga Toxin
  • Complement Factor H