title: The autoimmune lymphoproliferative syndrome (Canale-Smith) in adulthood.
abstract: The autoimmune lymphoproliferative syndrome (ALPS) or Canale-Smith syndrome is a recently described clinical entity consisting of chronic, non-malignant lymphadenopathy and hepatosplenomegaly together with hypergammaglobulinemia, positive autoantibodies and/or overt autoimmune diseases. It is caused by a genetic defect in the mechanism of programmed cell death (apoptosis) and is characterized by the presence of double-negative (TCR alpha/beta CD4- CD8-) T lymphocytes (DNT). Although well known in pediatric patients, ALPS is an unusual diagnosis in adults. The oldest reported patient was aged 54. We describe another two adult patients in whom a presenting autoimmune disease led to the diagnosis of ALPS.
authors.affiliation: Academic Department of Internal Medicine, Hippocration General Hospital, Athens, Greece.
authors.forename: M
authors.initials: M
authors.lastname: Deutsch
authors.forename: E
authors.initials: E
authors.lastname: Tsopanou
authors.forename: S P
authors.initials: SP
authors.lastname: Dourakis
mesh_terms: D000328:Adult; D017209:Apoptosis; D001323:Autoantibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008875:Middle Aged; D013577:Syndrome; D013601:T-Lymphocytes / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D005938:Glucocorticoids