Gamma heavy chain disease with an unusually benign course

Gamma heavy chain disease (gamma HCD) has been found in approximately 60 patients (Franklin 1978) since its discovery in 1964 (Franklin et al. 1964). Clinical features include fever and local or generalized lymphadenopathy which may wax and wane. Patients suffer from an undue susceptibility to infection. Hepatosplenomegaly is common. Blood smears often reveal lymphocytosis with atypical lymphocytes and eosinophilia. The pathological diagnosis is lymphoma or reactive hyperplasia. The disease is often rapidly progressive and fatal. In some patients it runs a more prolonged course. The case we present is remarkable in that the patient has now been in remission for 24 months.