title: Transitional cell carcinoma in a patient with X-linked hyperimmunoglobulin M syndrome.
abstract: Patients with X-linked hyperimmunoglobulin M syndrome (XHIGM) have a defective CD40-CD40 ligand system and further immunoglobulin class-switching. They may present with recurrent infection and malignancy involving the liver, pancreas or biliary tract. We report here a case of poorly differentiated transitional cell carcinoma in a young man with XHIGM even on regular treatment and discuss the possible pathogenesis. Given that the triggering of the CD40-CD40 ligand system has been found to improve tumor immunogenicity in recent studies, future immunotherapy targeting the CD40 ligand for these patients may be feasible to prolong their survival.
authors.affiliation: Department of Pediatrics, Saint Mary's Hospital, Luodong, Yilan, Taiwan.|Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan.
authors.forename: Jia-Shiuan
authors.initials: JS
authors.lastname: Li
authors.affiliation: Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan.
authors.forename: Shyh-Dar
authors.initials: SD
authors.lastname: Shyur
authors.affiliation: Department of Pathology, Changhua Christian Hospital, Changhua, Taiwan.
authors.forename: Ren-Hung
authors.initials: RH
authors.lastname: Huang
mesh_terms: D000328:Adult; D002295:Carcinoma, Transitional Cell / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D053307:Hyper-IgM Immunodeficiency Syndrome, Type 1 / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D007680:Kidney Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D008297:Male
keywords: CD40 ligand; X-linked hyper-IgM syndrome; malignancy; transitional cell carcinoma