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Table 3 Hematologic and Clinical Characteristics of endotheliopathy-associated DIT and true DIC

From: TTP-like syndrome: novel concept and molecular pathogenesis of endotheliopathy-associated vascular microthrombotic disease

  EA-DIT/VMTD and “DIC” of McKay True DIC
Example TTP-like syndrome APL
Nature of the clots “Microthrombi strings” made of platelet-ULVWF complexes “Fibrin clots” made of fibrin meshes
Mechanism of the genesis Intravascular microthrombogenesis Intravascular fibrinogenesis
Inciting causes/events Infection; surgery; pregnancy; transplant; cancer; drug; toxin, leading to edotheliopathy APL, leading to TF expression
Hematological manifestation Microthrombotic disorder Hemorrhagic disorder
Pathogenesis
 Mechanism Activation of microthrombotic pathway Activation of TF-initiated coagulation cascade
 Site of activation Intravascular membrane of ECs In circulation
 Thrombopathic result Intravascular hemostasis of ULVWF path Consumption of fibrinogen, FV and FVIII
Effect on the involved organ Hypoxic organ dysfunction Generalized bleeding tendency
Coagulation tests
 Fibrinogen Normal Decreased
 PT; aPTT; TT Normal Prolonged
 FVIII activity Normal or markedly increased Markedly decreased
 Thrombocytopenia Mild to moderately severe Not consumed but decreased due to APL
Associated clinical syndrome MODS; cytokine storm; SIRS Hemorrhagic syndrome
Associate hematologic features
 Schistocytes Often present Absent
 MAHA/aMAHA Almost always present Does not occur
 Hepatic coagulopathy Common Does not occur
Incidence in clinical practice Very common Extremely rare
Management
 Platelet transfusion Contraindicated May be used if needed for APL
 Treatment TPE; rADAMTS13 (expected to be very effective) Treat underlying pathology (e.g., ATRA in APL)
  1. APL acute promyelocytic leukemia, aPTT activated partial thromboplastin time; ATRA All-trans retinoic acid, DIC disseminated intravascular coagulation; DIT disseminated intravascular microhrombosis; FDP fibrin degradation products, FVIIa activated factor VII, FVIII factor VIII; MAHA/aMAHA microangiopathic hemolytic anemia/atypical MAHA, MODS, multi-organ dysfunction syndrome, PT, prothrombin time; rADAMTS13 recombinant ADAMTS13, SIRS systemic inflammatory response syndrome, TF tissue factor, TMA thrombotic microangiopathy; TPE therapeutic plasma exchange; TTP thrombotic thrombocytopenic purpura, ULVWF unusually large von Willebrand factor multimers; VMTD vascular microthrombotic disease