Lymphomatous proptosis as a novel feature of mantle cell lymphoma.

We describe eight cases of acute proptosis due to mantle cell lymphoma (MCL), among 26 consecutive MCL cases. The median time of onset was 29 months (range 0-102) from diagnosis. Two cases presented initially with orbital masses while five as sudden disease progression after multiple courses of chemotherapy. In each case, there was dramatic loss of vision and severe proptosis, which uniformly responded to radiotherapy and/or further chemotherapy. Unilateral blindness only occurred in two cases, with recurrent orbital relapse and repeated retinal irradiation and retro-orbital optic nerve involvement, respectively. The high incidence, as well as bilateral and recurrent nature, of orbital involvement suggested a homing mechanism of MCL to this site. Despite the absence of central nervous system involvement, most cases died of refractory disease. Apart from lymphomatous polyposis of the gut, MCL cells also show predilection to ocular presentation, and must be considered as a differential diagnosis to maltoma in the two anatomical sites.

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.

Vitiligo as a paraneoplastic syndrome preceding pituitary adenoma and subsequent acute lymphoblastic leukemia.

We present an unusual case of rapid onset of vitiligo heralding blindness due to pituitary apoplexy secondary to a non-secreting pituitary adenoma in a 49-year-old man. Deterioration in vision and skin condition coincided with tumor progression over 3 years. He subsequently died of acute lymphoblastic leukemia after pituitary radiation. The possible pathological associations between the 2 malignancies and vitiligo are discussed.