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