English

Abstract

BACKGROUND Ocular adnexa lympho-proliferative disorders are a divergent diverse category of ocular malignancies, comprising roughly about 1 % to 2 % of Non-Hodgkin’s lymphomas (NHLs) and 8 % of extranodal lympho-proliferative disorders. The most frequent type, approximating for up to 80 % of cases that constitutes the primary is marginal zone lymphoma of mucosa associated lymphoid tissue (MALT) type. Marginal zone lymphomas which usually have an extranodal presentation are routinely diagnosed by histomorphology by the diffuse infiltration of atypical lymphoid cells with plasmacytoid appearance and presence of pathognomonic lymphoepithelial lesions. Lymphoepithelial lesions are the existence of lymphocytes in the cytoplasm of epithelial cells. Immunohistochemistry shows a consistent absence of CD5 and CD23 staining, hence considered as diagnosis of exclusion on histo-immunomorphology. METHODS This is a case-series study. Twenty-two cases of ocular extranodal marginal zone lymphoma were obtained from the archives of pathology from 2013 to 2015. The histopathology and immunohistochemistry slides were reviewed by three expert histopathologists for confirmation of diagnosis. Immunohistochemical markers mainly used were CD45, CD3, CD5, CD20, cyclin D1, CD10, Ki - 67, BCL - 2, PAX 5 and CD23. The immunohistochemical markers such as CD10, BCL - 2 and cyclin D1 IHC expression were studied in cases of ocular extranodal marginal zone lymphoma (OENMZL). Relevant clinical details were collected from the patients such age, sex and history of autoimmune condition if any. RESULTS Eighteen cases (81 %) of OENMZL belonged to the age group of more than 40 years. There was a definite male preponderance (77 %) and it was associated with autoimmune conditions such as Hashimoto’s thyroiditis (18 %) and Sjogren’s syndrome (22 %). 22 cases of OENMZL were analysed and all showed consistent immunoexpression for CD20, CD45 while were immunonegative for CD5, CD23 and cyclin D1. 6 cases (27 %) showed CD10 positivity while 20 cases showed Bcl2 positivity (90 %). CONCLUSIONS OENMZL shows positivity for CD20 and CD45 while immunonegative for CD5, CD23 and cyclin D1, and a fraction of cases can show IHC positivity for CD10 and Bcl-2. KEYWORDS OENMZL: Ocular Extranodal Marginal Zone Lymphoma, MZL: Marginal Zone Lymphoma, CD: Cluster Differentiation. NHL: Non-Hodgkin Lymphoma. OL: Ocular Lymphoma. PCR: Polymerase Chain Reaction, FISH: Fluorescent In Situ Hybridization