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Table 1 Hematologic differential diagnosis amongst VMTD and similar coagulopathies

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

  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
  1. 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