Ocular Extranodal Marginal Zone Lymphoma - A Study of Twenty-Two Cases Presented at Bangalore, Karnataka

Abstract

Shankar Anand1, Akshatha C.2, Libin Babu Cherian3, Ramachandra C.4

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 Bcl-
2 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.
 

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