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

Table 1 Hematologic differential diagnosis amongst VMTD and similar coagulopathies

	GA-VMTD (hereditary TTP) AA-VMTD (autoimmune TTP)	EA-VMTD without HC (chronic “DIC”)	EA-VMTD with HC due to acute liver necrosis (acute “DIC”)	Fibrin clot disease (True DIC) (e.g., APL)	Primary fibrinolysis (e.g., amyloidosis)
Hematologic features
Thrombosis form	Microthrombi	Microthrombi	Microthrombi	Fibrin clots	NA
Hemostatic path	Aberrant ULVWF path	ULVWF path	ULVWF path	Aberrant TF path	NA
Thrombocytopenia	Always present	Variably present	Commonly present	Present due to APL	Not present
MAHA	Always present	Commonly present	Commonly present	Not present	Not present
MODS	Often present (brain and kidneys)	Often present	Often present	Not present	Not present
Acute liver failure	Uncommon (?)	Not present	Always present	Not present	?
Hemorrhage	Typically do not occur	Do not occur	Typically occur	Commonly occur	Persistent bleeding
Coagulation factors
Fibrinogen	Normal	Normal	Increased (early phase) decreased (late phase)	Decreased	Markedly decreased
FVIII	Normal	Markedly increased	Markedly increased	Markedly decreased	Decreased
FV	Normal	Normal	Decreased	Decreased	Normal to decreased
FVII	Normal	Normal	Markedly decreased	Normal	Normal
D-dimer/FDP	Normal	Normal	Increased	Increased	Increased
VWF/ULVWF	Normal	Markedly increased	Markedly increased	?	?
Coagulation tests
aPTT	Normal	Normal	Markedly prolonged	Markedly prolonged	Prolonged
PT	Normal	Normal	Markedly prolonged	Prolonged	Prolonged

Abbreviations: APL Acute promyelocytic leukemia, DIC Disseminated intravascular coagulation, DIT Disseminated intravascular microthrombosis, FDP Fibrin degradation products, MAHA Microangiopathic hemolytic anemia, aMAHA Atypical MAHA, MODS Multiorgan dysfunction syndrome, aPTT Activated partial thromboplastin time, PT Prothrombin time, TF Tissue factor, TT Thrombin time, TTP Thrombotic thrombocytopenic purpura, VWF Von Willebrand factor multimers, 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

ISSN: 1477-9560