title: Multicentric Castleman disease and systemic lupus erythematosus phenotype in a boy with Klinefelter syndrome: long-term disease stabilization with interferon therapy.
abstract: An 11-year-old boy with Klinefelter syndrome had Castleman disease (CD) of plasma cell type develop. Nonregulated antibody production mimicked systemic lupus erythematosus (SLE). Hepatitis C virus (HCV) infection caused significant disease worsening. The patient was treated with a daily dosage of 2 million units/m2 of IFN-alpha. Dramatic clinical improvement and decreasing autoimmune phenomenon were observed. HCV RNA were cleared. Hypergammaglobulinemia did not change. The boy has been living for 8 years with his disease. Plasma cell type CD can mimic collagenosis. Disease worsening is caused by HCV, though it can be reversed with IFN-alpha. Klinefelter syndrome may be a genetic susceptibility factor for CD in some cases.
authors.affiliation: Department of Pediatrics, Borsod County Teaching Hospital, Miskolc, Hungary.
authors.forename: R
authors.initials: R
authors.lastname: Simko
authors.forename: K
authors.initials: K
authors.lastname: Nagy
authors.forename: B
authors.initials: B
authors.lastname: Lombay
authors.forename: A
authors.initials: A
authors.lastname: Kiss
authors.forename: K
authors.initials: K
authors.lastname: Minik
authors.forename: V H
authors.initials: VH
authors.lastname: Lukacs
authors.forename: I
authors.initials: I
authors.lastname: Vamosi
mesh_terms: D005871:Castleman Disease / Q000188:drug therapy* / Q000209:etiology; D002648:Child; D006801:Humans; D007372:Interferons / Q000627:therapeutic use*; D007621:Karyotyping; D007713:Klinefelter Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D008180:Lupus Erythematosus, Systemic / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D010641:Phenotype; D017741:Survivors
chemical_list: D007372:Interferons