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Catastrophic antiphospholipid syndrome

ORPHA:464343· ICD-10 D68.6

Definition

A rare systemic autoimmune disease characterized by acute onset of life-threatening thromboses in three or more organs either simultaneously or within less than a week, in the presence of serum antiphospholipid antibodies (such as lupus anticoagulant, anticardiolipin antibodies, and anti-beta2-glycoprotein 1 antibodies), and with histopathological confirmation of small-vessel occlusion in at least one affected organ. The condition is often precipitated by infection, trauma, or surgery.

Prevalence
Unknown
Inheritance
Not applicable
Age of onset
Adult