title: Angioimmunoblastic lymphadenopathy with dysproteinemia: report of a case in infancy with review of literature.
abstract: A case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in infancy is reported. The disease had a mild onset with generalized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, polyclonal hypergammaglobulinemia, and T-cell deficiency. The AILD course lasted more than 100 months, alternating clinical remission to recurrent relapses. Hepatitis B viral infection suddenly evolving to hepatic failure was the cause of death. From a rapid survey of the present knowledge, the nosology, immunological features, and therapy of AILD are discussed and a possible presumptive pathogenetic pathway is proposed.
authors.affiliation: Department of 2nd Pediatrics, University of Bari, Italy.
authors.forename: M
authors.initials: M
authors.lastname: de Terlizzi
authors.forename: M G
authors.initials: MG
authors.lastname: Toma
authors.forename: T
authors.initials: T
authors.lastname: Santostasi
authors.forename: R
authors.initials: R
authors.lastname: Colella
authors.forename: A
authors.initials: A
authors.lastname: Ceci
authors.forename: G
authors.initials: G
authors.lastname: De Benedictis
mesh_terms: D001796:Blood Protein Disorders / Q000150:complications*; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000209:etiology / Q000276:immunology* / Q000628:therapy; D007167:Immunotherapy; D007223:Infant; D008297:Male; D013601:T-Lymphocytes / Q000276:immunology