The "catastrophic antiphospholipid syndrome (CAP) was first described by Acherson. It is a life-threatening disease with high mortality.
(1) presence of multiple vascular occlusions, especially involving small blood vessels (DIC)
(2) occurs after a triggering event (infection, trauma, obstetrical delivery, other)
(3) develops rapidly (fulminant)
(4) associated with antiphospholipid antibodies
(5) patients often develop the systemic inflammatory response syndrome (SIRS) with multiple organ failures
(6) thrombocytopenic and microangiopathic anemia common
Patients at risk:
(2) lupus-like disease
(3) primary antiphospholipid syndrome
Common causes of death:
(1) multi-organ failure
(2) cerebral involvement
(3) cardiac involvement
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Specialty: Hematology Oncology, Clinical Laboratory, Immunology/Rheumatology