Lymphocytic infiltrates of the orbit and conjunctiva may be divided into three his-tologic groups:
1. Monomorphous infiltrates of clearly atypical lymphocytes
2. Infiltrates composed of small lymphocytes with minimal or no cytologic atypia
3. Benign inflammatory pseudotumor or reactive follicular hyperplasia.
On the basis of immunophenotypic criteria, they can be divided into two classes:
1. Infiltrates with monotypic immunoglobulin expression
2. Infiltrates with polytypic immunoglobulin expression.
For the localized small lymphocytic infiltrates, monotypic (monoclonal) immuno-globulin expression confers a 50% risk of dissemination. The initial immunopheno-typic (mono- or polyclonal) and molecular studies of various histologic groups fail to correlate with the eventual outcome of these cases because the initial polyclonal tumors may become monoclonal.
All patients presenting with small lymphocyte infiltrates of the orbit and conjunctiva should have a systemic evaluation with serum chemistries, blood counts, and appropriate imaging studies at initial diagnosis and every 6 months for 5 years thereafter.
For localized disease, local radiotherapy is commonly used, regardless of histo-logic grading.
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