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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)