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S Raichand, A Weatherwax, G Lissner, D Variakojis, P Bryar; Conjunctival and Orbital Lymphoma: Clinical Course, Histopathology, Immunocytochemisty, and Gene Rearrangements . Invest. Ophthalmol. Vis. Sci. 2002;43(13):2586.
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Purpose: To describe characteristics of lymphoid lesions of the orbit and conjunctiva with histopathology, imunocytochemisty, and gene rearrangement studies. Method: This is a retrospective study of 27 patients diagnosed with conjunctival, eyelid, or orbital lymphoid lesions. We reviewed and compiled data on all patients in which a biopsy was done over the last 18 years. This included clinical presentation, diagnosis, treatment and outcome. Paraffin tissue blocks were reviewed to confirm prior reading and/or to reclassify the lesion. Flow cytometry and gene rearrangement studies were done if tissue sample allowed. Results: Clinical presentation included: mass or swelling of the ocular adenexa (55%), ptosis (19%), proptosis (11%), incidental (11%), and decreased vision (4%). Twenty-three patients (85%) had unilateral disease. Sites of involvement were: orbit (60%), orbit and lacrimal sac (22%), conjunctiva (11%), lid (4%) and lacrimal sac (4%). At the time of diagnosis five of the 27 patients (19%) had systemic lymphoma. Histopathology revealed malignant lymphoma in 23 of 27 patients (86%), atypical lymphoid hyperplasia in 2 (7%), and indeterminate lymphoma in 2 (7%). Immunophenotypic studies were done if possible. Twenty of 20 were positive for CD20, 16 of 18 were positive for CD3, and one of 16 was positive for Ki-67. Flow cytometry revealed B cell predominance in 38%, T cell predominance in 12%, and mixed B and T cells in 50%. Light chain restriction was present in 75% of the samples. Paraffin tissue blocks could be obtained on 11 of the 27 patients. Genetic studies revealed IgH rearrangement in 9 of 11 (81%) patients. Of the 27 patients with ocular adnexal lymphoid lesions 8 (30%) had or eventually developed systemic lymphoma. Conclusion: The majority of lymphoid lesions of the orbit and conjunctiva are a mixture of B and T cells. Most of the lymphomatous biopsies tested (81%) were positive for the IgH rearrangement. Of the lymphomas with light chain restriction, half had or eventually developed systemic lymphoma. Flow cytometry and gene rearrangement studies are valuable tools in evaluating ocular lymphoid lesions.
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