High-dose gamma-globulin therapy in the reactive haemophagocytic syndrome

Summary. Reactive haemophagocytic syndrome (RHS) is a disorder characterized by systemic proliferation of non-malignant histiocytes occuring most commonly in patients with pre-existing immunological abnormalities or neoplasma. Patients, particularly those with immunosuppression, often have a rapidly progressive fatal course. Treatment is directed at the underlying disorder. In the absence of identifiable cause, the therapy is less satisfactory. We report here three cases of RHS successfully treated with high-dose gamma-globulin therapy. Two of the three patients were immunocompromised and the third occurred during pregnancy. The improvement occurred within 24–72 h and all patients recovered. High-dose i.v. gamma-globulin therapy may be beneficial in RHS.