Table 1 Comparison of catastrophic... | American Society of Hematology

Table 1

Comparison of catastrophic thromboembolic disorders

	Catastrophic APS	Atypical TTP	Cancer-associated thrombosis	Delayed or spontaneous HIT	“Idiopathic” catastrophic thrombosis
Typical precipitating events/clinical conditions	Infection, surgery, trauma, pregnancy	Pancreatitis, surgery, infection, pregnancy	Cancer (known or unknown at presentation)	Surgery, infections; recent prior heparin exposure with delayed HIT	Infection, surgery, trauma, pregnancy
Thrombotic phenotype	Microvascular events dominate; large vessel occlusions also occur	Arterial events may precede hematologic findings	Superficial and deep venous thrombosis; arterial thrombosis	Arterial and venous thrombotic events	Arterial, venous, and/or microvascular events
Laboratory findings	Lupus anticoagulant, anticardiolipin antibodies, anti-β₂glycoprotein I antibodies	Decreased ADAMTS13 level; schistocytes; thrombocytopenia	Associated with DIC; cancer-specific markers may be present	Platelet-activating antibodies to PF4/heparin; thrombocytopenia	No specific diagnostic tests
Primary therapy	Anticoagulation, plasma exchange, corticosteroids	Plasma exchange	Anticoagulation, especially LMWH	Anticoagulation with nonheparin agents	Anticoagulation
Alternative/additional therapies	Cyclophosphamide, rituximab, IVIG	Immunosuppression	Therapy for the underlying malignancy	Plasma exchange has been used for “refractory” HIT	Antiplatelet therapy may be useful for arterial events

	Catastrophic APS	Atypical TTP	Cancer-associated thrombosis	Delayed or spontaneous HIT	“Idiopathic” catastrophic thrombosis
Typical precipitating events/clinical conditions	Infection, surgery, trauma, pregnancy	Pancreatitis, surgery, infection, pregnancy	Cancer (known or unknown at presentation)	Surgery, infections; recent prior heparin exposure with delayed HIT	Infection, surgery, trauma, pregnancy
Thrombotic phenotype	Microvascular events dominate; large vessel occlusions also occur	Arterial events may precede hematologic findings	Superficial and deep venous thrombosis; arterial thrombosis	Arterial and venous thrombotic events	Arterial, venous, and/or microvascular events
Laboratory findings	Lupus anticoagulant, anticardiolipin antibodies, anti-β₂glycoprotein I antibodies	Decreased ADAMTS13 level; schistocytes; thrombocytopenia	Associated with DIC; cancer-specific markers may be present	Platelet-activating antibodies to PF4/heparin; thrombocytopenia	No specific diagnostic tests
Primary therapy	Anticoagulation, plasma exchange, corticosteroids	Plasma exchange	Anticoagulation, especially LMWH	Anticoagulation with nonheparin agents	Anticoagulation
Alternative/additional therapies	Cyclophosphamide, rituximab, IVIG	Immunosuppression	Therapy for the underlying malignancy	Plasma exchange has been used for “refractory” HIT	Antiplatelet therapy may be useful for arterial events