Table 2.

Diagnosing the pathophysiology of thrombocytopenia-associated multiple organ failure

	Diagnostic criteria	Treatment
TTP	Fever	Steroids for 24 hours
	Thrombocytopenia	Within 30 hours perform 1 1/2 volume plasma exchange then 1 volume daily until resolution of thrombocytopenia (median 18 days [18])
	Increased LDH
	Schistocytes >5%	If recalcitrant use cryopreserved supernatant
	Neurological and renal dysfunction	If continues at 28 days use vincristine
DIC	Thrombocytopenia	Reverse shock and underlying disease (increase flow with fluids and consider vasodilators – nitroglycerin, milrinone, pentoxyfilline)
	Decreased factors V and X, and fibrinogen
	Decreased antithrombin III and protein C	Replace clotting factors with FFP, cryoprecipitate and platelets via plasma infusion or plasma exchange
	Increased D-dimers
	Prolonged PT/aPTT	Anticoagulate with heparin, protein C, activated protein C, antithrombin III, or prostacyclin
		Use fibrinolytics for life or limb threatening thrombosis. Remember to keep PT/aPTT and platelets normal when giving fibrinolytics
		Give anti-fibrinolytics if life threatening bleeding (rarely needed when PT/aPTT and platelet counts are maintained)
Secondary TMA	Thrombocytopenia	Remove source of secondary TMA
	Increased LDH	Activated protein C for adult severe sepsis [26]
	Normal or elevated fibrinogen	TTP based plasma exchange (median 9 days [51]; median 12 days for children (Nguyen, 2006, submitted)
	<5% schistocytes
	Multiple organ failure

aPTT, activated partial thromboplastin time; DIC, disseminated intravascular coagulation; FFP, fresh frozen plasma; LDH, lactate dehydrogenase; PT, prothrombin time; TMA, thrombotic microangiopathy; TTP, thrombotic thrombocytopenic purpura.