Table 2.
1—Genesis of Primary Phenotypes of Thrombosis |
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(1) The phenotype is determined by the functional character of the injured vessel (i.e., vascular pressure and oxygen/CO2 carrying function: arterial or venous vasculature) (2) The phenotype is determined by the location of the involved vessel (i.e., involved organ or tissue: brain, lungs, heart, liver, nerve, muscle etc.) (3) The phenotype is determined by the size of the vasculature (i.e., microvasculature and larger vessel: capillary/arteriole, venule, artery, vein) (4) The phenotype is determined by the depth of vascular wall injury (i.e., ECs, SET, and/or EVT) |
2—Genesis of Secondary Phenotype of Thrombosis |
(1) The secondary phenotype is influenced by the extent of vascular tree involvement (e.g., localized injury vs. disseminated injury: e.g., sepsis vs. local trauma) (2) The secondary phenotype is influenced by the underlying genetic disorder (e.g., thrombophilia: e.g., ADAMTS13 deficiency, protein C deficiency, FV-Leiden) (3) The secondary phenotype is influenced by the iatrogenic vascular injury (e.g., hospitalization related vascular injury such as surgery, and vascular device/access) (4) The secondary phenotype is influenced by inappropriate treatment (e.g., platelet transfusion [e.g., for ITP], or recombinant FVIIa treatment causing fibrin clot disease) |
3—Basic and Combined Phenotypes of Thrombosis |
(1) Microthrombosis, macrothrombosis, fibrin clot disease, and hematoma a (e.g., EA-VMTD, TTP-like syndrome, MODS, AT, ITP, isolated DVT, isolated PT, APL, hemarthrosis a) (2) Multiple combined micro-macrothrombosis (i.e., micro-macrothrombosis with gangrene: e.g., SPG, PDIS, ANF, acrocyanosis, Fournier’s disease; micro-macrothrombosis with VCCS: e.g., VTE, PTE, CVST) (3) Complex phenotypes with underlying VMTD and additional localized vascular injury and genetic thrombophilia (e.g., various phenotypes of “DIC” with microthrombosis and hepatic coagulopathy with/without thrombophilia, Kawasaki disease with inflammation, purpura fulminans) |
Note: APL, acute promyelocytic leukemia; ANF, acute necrotizing fasciitis; AT arterial thrombosis; VCCS, venous circulatory congestion syndrome; CVST, cerebral venous sinus thrombosis; “DIC”, “false” disseminated intravascular coagulation; DVT, deep vein thrombosis; EA-VMTD, endotheliopathy-associated vascular microthrombotic diseases; ECs, endothelial cells; EVT, extravascular tissue; ITP, immune thrombocytopenic purpura; IVCT, inferior vena cava thrombosis; MODS, multiorgan dysfunction syndrome; PDIS, peripheral digit ischemic syndrome; PT, pulmonary thrombosis; PTE, pulmonary thromboembolism; SET, subendothelial tissue; SPG, symmetrical peripheral gangrene; TTP, thrombotic thrombocytopenic purpura; VTE, venous thromboembolism; a indicates extravascular blood clots.