N2010 adult-onset still's disease complicated by hemophagocytic syndrome and catastrophic antiphospholipid syndrome resulting in four limb amputation

摘要

Hemophagocytic syndrome is a potentially fatal hyperinflammatory condition, characterized by aggressive proliferation of activated macrophages and histio-cytes that phagocytose other hemopoeitic cells. This overactive immune reaction is responsible for the clinical manifestations of HPS [1], namely prolonged fever, hepa-tosplenomegaly, lymphadenopathy, skin rash, central nervous system abnormalities, pancytopenia and coagulation disorders. HPS has been classically subdivided into primary or genetic, and secondary or acquired, although more recently this distinction has been thought to be an oversimplification. The secondary type may be associated with autoimmune diseases (when it is sometimes referred to as mac-rophage-activation syndrome), specifically systemic juvenile idiopathic arthritis and adult-onset Stills disease; viral infection, most commonly Epstein-Barr virus; and underlying malignancy [1].