Interferon-alpha activity in a case of severe autoimmune lymphoproliferative disease.
Autoimmune lymphoproliferative disease (ALD) is a rare familial disorder. Clinical and laboratory features of this disease include a generalized lymphadenopathy, splenomegaly, increased levels of circulating CD3+ with low levels of CD4+, CD8+ T-cells, and autoimmune phenomena, characteristics that the 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 unresponsive to cyclosporine. Nevertheless, he was successfully treated with interferon (IFN)-alpha, resulting in a long-lasting, clinically complete remission.
Ceretelli
S
S
Department of Oncology, University of Pisa, Italy.
Petrini
M
M
Galimberti
S
S
Testi
C
C
Frizzera
G
G
D000293:Adolescent; D001327:Autoimmune Diseases / Q000188:drug therapy*; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D008232:Lymphoproliferative Disorders / Q000188:drug therapy* / Q000276:immunology; D008297:Male
D016898:Interferon-alpha