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

Table 4 Hematologic differential diagnoses among thrombopathies and coagulopathies

	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 Treatment	Petechiae; Usually no need of treatment	May cause serious bleeding; Controllable with FFP & rFVIIa	Common, serious bleeding; Abrogated with ATRA & chemotherapy	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

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

ISSN: 1477-9560