A Case of Thrombotic Thrombocytopenia Purpura Associated with Systemic Lupus Erythematosus: Diagnostic Utility of ADAMTS-13 Activity

Table 2

Differential diagnosis of hemolytic anemia with thrombocytopenia. The diseases possibly resembling TTP are summarized in Table 1. TTP: thrombotic thrombocytopenic purpura, HUS: hemolytic-uremic syndrome, CAPS: catastrophic anti-phopholipid antibody syndrome, DIC: disseminating intravascular coagulopathy.

	TTP	HUS	EVANS syndrome	CAPS	DIC
Tthrombocytopenia	(+)	(+)	(+)	(+)	(+)
Hemolytic anemia	(+)	(+)	(+)	(+−)	(+−)
Sschistocytes	(++)	(+)	(+−)	(+−)	(+−)
Clinical findings	Mental disorder	Diarrhea	Autoimmue reaction	Multiple organ infarction	Underlying disease
	Renal dysfunction	Renal dysfunction
	Fever up
Specific laboratory findings	Suppression of ADAMTS-13 activity	Positive for Shiga-toxin	Positive for Coombs test	Antiphospholipid antibodies	Elevation of FDP
Ttreatment	Plasma exchange	Hydration	Steroids	Anti-coagulant therapy	Anti-coagulant therapy
	Steroids	Plasma exchange	Immunosuppression therapy (IVCY)	Steroids	Treatment for underlying disease
	1mmunosuppression therapy(IVCY)			Iimmunosuppression therapy (IVCY)
	Anti-coagulant therapy			Plasma exchange