Table 6.
Pathogenetic features of combined micro-macrothrombosis in arterial and venous systems per “two-path unifying theory” of hemostasis.
| Clinical Phenotype | Arterial Combined Micro-Macrothrombosis | Venous Combined Micro-Macrothrombosis |
|---|---|---|
| Pathologic nature of thrombosis | ||
| (Examples) | Gangrene: | Venous circulatory congestion syndrome: |
| SPG, limb gangrene, Fournier’s disease, PF, diabetic gangrene, necrotizing fasciitis |
Proximal/central DVT, VTE, PTE, CVST, PVT, IVCT/SVCT, SVT, Budd-Chiari syndrome | |
| Primary event | ||
| Vascular injury (ECs) | Diseases causing microvascular endotheliopathy (e.g., sepsis, diabetes, pregnancy) |
Diseases causing venous endotheliopathy (e.g., sepsis, diabetes, pregnancy, autoimmunity) |
| Vascular pathology site | Disseminated aEA-VMTD at terminal arterial tree | Regional vEA-VMTD at vascular injury site |
| Activated hemostatic path | ULVWF path | ULVWF path |
| Thrombosis component | Microthrombi strings | Microthrombi strings |
| Secondary event | ||
| Vascular injury (SET) | Arterial vascular damage (e.g., surgery, vascular accesses/devices) |
Venous vascular damage (e.g., surgery, vascular accesses/devices) |
| Pathology | Fibrin clots in arterial system | Fibrin clots in venous system |
| Vascular pathology site | Terminal arterial vasculature tree | Large vein or pulmonary artery |
| Activated hemostatic path | TF path | TF path |
| Thrombosis component | Fibrin meshes | Fibrin meshes |
| Pathogenesis | ||
| Mechanism | Unifying of microthrombi string and fibrin meshes in arterial system as minute macrothrombi | Unifying of microthrombi strings and fibrin meshes in venous system often as connected macrothrombi |
| Thrombosis character | Microthrombi strings-fibrin meshes complex | Microthrombi strings-fibrin meshes complex |
| Thrombosis form and effect | Multiple terminal efferent digit anoxia and peripheral gangrene | Connected and regional, with circulatory afferent flow with venous circulatory congestion syndrome |
Note: aEA-VMTD, arterial endotheliopathy-associated vascular microthrombotic disease; vEA-VMTD, venous-VMTD; CVST, cerebral venous sinus thrombosis; ECs, endothelial cells, DVT, deep venous thrombosis; IVCT, inferior vena cava thrombosis; PF, purpura fulminans; PVT, portal vein thrombosis; SET, subendothelial issue; SPG, symmetrical peripheral gangrene; SVCT, superior vena cava thrombosis; SVT, splanchnic vein thrombosis; TF, tissue factor; PTE, pulmonary thromboembolism; ULVWF, ultra large von Willebrand factor: VTE, venous thromboembolism.