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.