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Table 2 Pathogenetic difference of VMTD between TTP and sepsis-associated TTP-like syndrome

From: Sepsis and septic shock: endothelial molecular pathogenesis associated with vascular microthrombotic disease

  TTP (GA-VMTD and AA-VMTD) TTP-like syndrome in sepsis (EA-VMTD)
Primary event Hereditary TTP (GA-VMTD) Sepsis due to pathogens
(due to mutation of ADAMTSD13 gene) (e.g., bacteria; viruses; fungi; rickettsia; parasites)
Acquired TTP (AA-VMTD)
(due to anti-ADAMTS13 antibody) Complement activation → MAC (C5b-9) formation (?)
Secondary event Excess of circulating ULVWF & platelets C5b-9-induced endotheliopathy
Microthrombogenesis, leading to ULVWF-platelet complex formation in microcirculation ULVWF released from ECs & anchored to endothelial membrane to recruit platelets → microthrombogenesis
Tertiary event Microthrombi in microvasculatures Microthrombi strings in smaller and larger vasculatures
Final event Microvascular thrombosis Vascular microthrombosis
TTP TTP-like syndrome
  1. Abbreviations: ECs endothelial cells, MAC membrane attack complex, TTP thrombotic thrombocytopenic purpura, VMTD vascular microhrombotic disease, AA-VMTD antibody-associated VMTD, EA-VMTD endotheliopathy-associated VMTD, ULVWF unusually large von Willebrand factor multimers