Interferon-alpha activity in a case of severe autoimmune lymphoproliferative disease

Autoimmune lymphoproliferative disease (ALD) is a rare familial disorder. C linical and laboratory features of this disease include a generalized lymph adenopathy, splenomegaly, increased levels of circulating CD3+ with low lev els of CD4+, CD8+ T-cells, and autoimmune phenomena, characteristics that t he autoimmune lymphoproliferative syndrome (ALPS) have in common. Treatment usually consists of different supportive therapies. We report on the case of a young man affected by ALD who became resistant to steroids and was unr esponsive to cyclosporine. Nevertheless, he was successfully treated with i nterferon (IFN)-alpha, resulting in a long-lasting, clinically complete rem ission.