title: High-grade lymphoma after azathioprine treatment for Vogt-Kaganayi-Harada syndrome.
abstract: Vogt-Kaganayi-Harada (VKH) syndrome is a rare autoimmune disease characterized by panuveitis, neuropathy and aseptic meningitis. Most patients require long-term treatment with steroids and immunosuppressants. Patients may develop concurrent autoimmune diseases, especially endocrinopathies. Secondary malignancies are rare associations. We report a Chinese man with VKH syndrome presenting with multiple cranial nerve palsy and bilateral pan-uveitis, who developed disseminated high-grade B cell lymphoma after 3-year treatment with azathioprine. This is the first report of systemic non-Hodgkin lymphoma in patients with VKH syndrome. The carcinogenic properties of azathioprine on an abnormally expanded but non-clonal lymphoid system may play a role in the pathogenesis.
authors.affiliation: Department of Ophthalmology, Queen Mary Hospital, the University of Hong Kong, Hong Kong.
authors.forename: Charmaine
authors.initials: C
authors.lastname: Hon
authors.forename: Shu-Leong
authors.initials: SL
authors.lastname: Ho
authors.forename: Edmond S K
authors.initials: ES
authors.lastname: Ma
authors.forename: Nigel J
authors.initials: NJ
authors.lastname: Trendell-Smith
authors.forename: Wing Y
authors.initials: WY
authors.lastname: Au
mesh_terms: D000328:Adult; D001379:Azathioprine / Q000009:adverse effects*; D002273:Carcinogens / Q000009:adverse effects; D003389:Cranial Nerve Diseases / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D016130:Immunophenotyping; D008198:Lymph Nodes / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000139:chemically induced / Q000209:etiology; D008228:Lymphoma, Non-Hodgkin / Q000139:chemically induced* / Q000175:diagnosis / Q000209:etiology; D008297:Male; D014605:Uveitis / Q000175:diagnosis / Q000209:etiology; D014607:Uveomeningoencephalitic Syndrome / Q000150:complications* / Q000188:drug therapy
chemical_list: D002273:Carcinogens; D001379:Azathioprine