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Table 4 Classification of thrombotic disorder based on genesis of intrinsic characters of “blood clots”

From: Disseminated intravascular coagulation: new identity as endotheliopathy-associated vascular microthrombotic disease based on in vivo hemostasis and endothelial molecular pathogenesis

  Microthrombosis Fibrin clot disease Macrothrombosis
Level of vascular injury ECs (Level 1) EVT (Level 2 only) ECs/SET/EVT (Combined Level 3)
Activated path ULVWF or aberrant ULVWF path TF or aberrant TF path Combined ULVWF & TF path
Mechanism Microthrombogenesis Fibrinogenesis Macrothrombogenesis
Participants ULVWF
Platelet
TF
Coagulation factors
ULVWF-platelet complexes
Fibrin meshes
NETs
Character of thrombi Microthrombi Fibrin clots Macrothrombus
Examples of disease VMTD
Generalized: EA-VMTD (e.g., TTP-like syndrome, including false DIC)
AA-VMTD*
GA-VMTD*
Local: Kasabach-Merritt syndrome
Focal: TIA; HERNS syndrome
Hemorrhagic stroke: SDH
Hemarthrosis
APL-associated coagulopathy*
DVT
PE
Myocardial infarction
Acute ischemic stroke
Gangrene
  1. Abbreviations: APL Acute promyelocytic leukemia, DIC Disseminated intravascular coagulation, “DIC”, False DIC, DVT Deep vein thrombosis, HERNS Hereditary endotheliopathy, retinopathy, nephropathy and stroke syndromes, MI Myocardial infarction, NETs Neutrophil extracellular traps, PE Pulmonary embolism, SDH Subdural hematoma, TIA Transient ischemic attack, TF Tissue factor, ULVWF Unusually large von Willebrand factor multimers, VMTD Vascular microthrombotic disease, AA-VMTD Antibody-associated VMTD, EA-VMTD Endotheliopathy-associated VMTD. GA-VMTD, gene mutation-associated VMTD. AA-VMTD*, GM-VMTD* and true DIC* in APL do not occur due to vascular injury, but utilizes hemostatic paths: aberrant ULVWF path or aberrant TF path (please see the text)