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Pathology

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In TTP there is aggregation of platelets that obstruct reversibly the arterioles and capillaries of various organs resulting in fluctuating ischaemia (and sometimes infarction).

Features include:

  • brain is involved in 50-71% of cases
  • early capillary and arteriolar microthrombi in TTP consist almost exclusively of microthrombi without perivascular inflammation
  • TTP is associated with excessive release of ULvWF multimeric forms from systemic endothelial cells. If these endothelial cells are perturbed transiently then a single-episode of TTP occurs; if perturbed periodically then intermittent TTP occurs
  • immunohistochemical studies in thrombi in TTP have revealed:
    • an abundance of von Willebrand factor
    • little fibrinogen/fibrin
    • both are opposite findings to those seen in thrombotic lesions in DIC

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