Increased levels of vascular endothelial cell markers in thrombotic thrombocytopenic purpura

Am J Hematol. 1993 Oct;44(2):101-5. doi: 10.1002/ajh.2830440206.


We found that patients with thrombotic thrombocytopenic purpura (TTP) have significantly elevated plasma thrombin antithrombin III complex (TAT) and FDP-D-dimer levels, while the plasmin-alpha 2 plasmin inhibitor complex (PIC) level was only slightly increased. The tissue-type plasminogen activator (t-PA) level was increased, but it was well correlated with the plasminogen activator inhibitor-I (PAI-I) level. These findings suggest that hypercoagulable and hypofibrinolytic states coexist in these patients, in contrast to patients with disseminated intravascular coagulation, who exhibit coexisting hypercoagulable and hyperfibrinolytic states. Levels of vascular endothelial cell markers, such as PAI-I, thrombomodulin (TM), and t-PA, were increased at the onset of TTP, but the level of von Willebrand factor (vWF) antigen was not increased. The outcome in TTP patients was correlated with plasma t-PA and TM levels but not with TAT or PIC. These results suggest that vascular endothelial cell markers, such as TM and t-PA, are released from injured or stimulated endothelial cells, reflecting the degree of vascular endothelial damage, and that the main factor in the pathogenesis of TTP is vascular endothelial cell injury.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antifibrinolytic Agents / analysis
  • Antithrombin III / analysis
  • Endothelium, Vascular / chemistry*
  • Endothelium, Vascular / pathology*
  • Female
  • Fibrin Fibrinogen Degradation Products / analysis
  • Fibrinolysin / analysis
  • Hemostasis
  • Humans
  • Male
  • Middle Aged
  • Plasminogen Activator Inhibitor 1 / analysis*
  • Plasminogen Activator Inhibitor 1 / blood
  • Prognosis
  • Purpura, Thrombotic Thrombocytopenic / blood*
  • Purpura, Thrombotic Thrombocytopenic / pathology
  • Thrombomodulin / analysis*
  • Tissue Plasminogen Activator / analysis*
  • Tissue Plasminogen Activator / blood
  • alpha-2-Antiplasmin*
  • von Willebrand Factor / analysis


  • Antifibrinolytic Agents
  • Fibrin Fibrinogen Degradation Products
  • Plasminogen Activator Inhibitor 1
  • Thrombomodulin
  • alpha-2-Antiplasmin
  • plasmin-plasmin inhibitor complex
  • von Willebrand Factor
  • Antithrombin III
  • Tissue Plasminogen Activator
  • Fibrinolysin