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Table 2 Characteristics of two different ULVWF multimers

From: TTP-like syndrome: novel concept and molecular pathogenesis of endotheliopathy-associated vascular microthrombotic disease

  mULVWF multimers eULVWF multimers
Synthesized in Megakaryocytes Endothelial cells
Stored in α granules of platelets Weibel-Palade bodies of ECs
Primary distribution at release In circulation On the membrane of ECs
Availability In microcirculation At ECs following endothelial exocytosis
Exposure to ADAMTS13 As platelet-adherent form As ECs-adherent form
Interaction with platelets causing Platelet aggregation and adhesion Platelet-ULVWF strings
Localization of platelet-ULVWF complexes Arteriolar and capillary lumens lodged as microthrombi in situ Endothelial membrane-anchored as microthrombi strings
Example of leading its activity ADAMTS13 autoantibody Sepsis-induced endotheliopathy
Endotheliopathic lesion Microthrombotic microangiopathy Microthrombotic angiopathy
Hematologic manifestation Thrombocytopenia and MAHA Thrombocytopenia and MAHA/aMAHA
Associated inflammation None to minimal (?) Mild to severe
Associated clinical syndrome TTP TTP-like syndrome
  1. ECs endothelial cells, eULVWF/mULVWF endothelial unusually large von Willebrand factor/megakaryocytic ULVWF, MAHA/aMAHA microangiopathic hemolytic anemia/atypical MAHA, TTP thrombotic thrombocytopenic purpura