[Thrombotic microangiopathies]

Ugeskr Laeger. 2009 Nov 30;171(49):3614-20.
[Article in Danish]

Abstract

The thrombotic microangiopathic diseases, which include acquired and congenital TTP and HUS, are most frequently acute disease entities. Untreated, these diseases are associated with a lethal course in many cases. Deficiency of the von Willebrand cleaving enzyme, ADAMTS13, is a decisive pathophysiological defect in most cases of thrombotic microangiopathic diseases. Early recognition and a secure distinction from other conditions that resemble TMA diseases is essential in order to institute adequate treatment and to obtain a favourable outcome in terms of both survival and development of serious sequelae.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Diagnosis, Differential
  • Early Diagnosis
  • Hemolytic-Uremic Syndrome / diagnosis
  • Hemolytic-Uremic Syndrome / mortality
  • Hemolytic-Uremic Syndrome / therapy
  • Humans
  • Prognosis
  • Purpura, Thrombocytopenic / diagnosis
  • Purpura, Thrombocytopenic / mortality
  • Purpura, Thrombocytopenic / therapy
  • Purpura, Thrombotic Thrombocytopenic / diagnosis
  • Purpura, Thrombotic Thrombocytopenic / mortality
  • Purpura, Thrombotic Thrombocytopenic / therapy
  • Thrombotic Microangiopathies* / diagnosis
  • Thrombotic Microangiopathies* / mortality
  • Thrombotic Microangiopathies* / therapy