title: Treatment of X-linked lymphoproliferative disease (Duncan disease) with high-dose methylprednisolone and etoposide (VP-16).
abstract: A five-year-old boy in the acute phase of X-linked lymphoproliferative (XLP) syndrome (Duncan disease) with high fever and hepatosplenomegaly was treated successfully with high-dose methylprednisolone and VP-16 for 15 months. He had been alive for four years after diagnosis as of this writing. We recommend high-dose methylprednisolone and VP-16 in patients with XLP who have to wait for a suitable donor before bone marrow transplantation.
authors.affiliation: Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara.
authors.forename: A
authors.initials: A
authors.lastname: Gürgey
authors.forename: T
authors.initials: T
authors.lastname: Sayli
authors.forename: A
authors.initials: A
authors.lastname: Kara
authors.forename: G
authors.initials: G
authors.lastname: Kale
authors.forename: I
authors.initials: I
authors.lastname: Berkel
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use*; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005047:Etoposide / Q000627:therapeutic use*; D006801:Humans; D007223:Infant; D008232:Lymphoproliferative Disorders / Q000188:drug therapy* / Q000235:genetics; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use*; D010375:Pedigree; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D000972:Antineoplastic Agents, Phytogenic; D005047:Etoposide; D008775:Methylprednisolone