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Table 4 Hematologic differential diagnoses among thrombopathies and coagulopathies

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

  TTP & TTP-like syndrome (DIT) TTP-like syndrome (DIT) associated with HC (e.g., sepsis) equal to acute “DIC” DIC (e.g., APL) PF (e.g., amyloidosis)
Thrombocytopenia Always present Always present Present due to APL, but not due to consumption (?) Not present
MAHA/aMAHA Always present Always present Do not occur Not present
Fibrinogen Normal Decreased Always decreased Always decreased
Factor VIII Normal Normal or increased Markedly decreased Decreased
Factor V Normal Decreased Decreased Normal or decreased
Factor X Normal Decreased Usually normal Normal (?)
Factor VII Normal Markedly decreased Normal Normal
Factor IX Normal Decreased Normal Normal
FDP Normal ? Positive Strongly positive
Prothrombin time Normal Prolonged Prolonged Prolonged
Activated partial
thromboplastin time
Normal Prolonged Prolonged Prolonged
Thrombin time Normal Prolonged Prolonged Prolonged
Thrombosis form Microthrombi Microthrombi Friable fibrin clots (meshes) Absent
Bleeding: Character
Usually no need of treatment
May cause serious bleeding;
Controllable with FFP & rFVIIa
Common, serious bleeding;
Abrogated with ATRA &
Slow & persistent bleeding;
Treatable with AFA
Hypoxic organ
dysfunction (MODS)
Present Present Not present Not present
Platelet transfusion Contraindicated Contraindicated May be used for APL Not needed
  1. AFA anti-fibrinolytic agent, ATRA all-trans retinoic acid, “DIC” false disseminated intravascular coagulation, DIT disseminated intravascular microthrombosis, FDP fibrin degradation products, FFP fresh frozen plasma; HC hepatic coagulopathy; MAHA/aMAHA microangiopathic hemolytic anemia/atypical MAHA, PF primary fibrinolysis, TTP thrombotic thrombocytopenic purpura