High-grade lymphoma after azathioprine treatment for Vogt-Kaganayi-Harada syndrome.
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.
Hon
Charmaine
C
Department of Ophthalmology, Queen Mary Hospital, the University of Hong Kong, Hong Kong.
Ho
Shu-Leong
SL
Ma
Edmond S K
ES
Trendell-Smith
Nigel J
NJ
Au
Wing Y
WY
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
D002273:Carcinogens; D001379:Azathioprine