Reactive Hemophagocytic Syndrome

Abstract

Two cases o f reactive hemophagocytic syndrome (RHS) are reported, and the clinical and pathological features are compared with other histiocytic proliferative disorders, including familial hemophagocytic lymphohistiocytosis (FHL) and malignant histiocytosis. RHS can be associated with a variety o f infections, including viral, bacterial, fungal, and parasitic. RHS may also be familial as exemplified by our 2 cases in siblings. The isolation of an effective agent appears t o be the only criterion by which a diagnosis o f RHS can be made.

Key Words:

• reactive hemophagocy tic histiocytosis
• lymphohistiocy tic proliferative disorder
• virus-associated hemophagocytic syndrome
• infection-associated hemophagocy tic syndrome