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Blood, 16 July 2009, Vol. 114, No. 3, pp. 501-510. Prepublished online as a Blood First Edition Paper on April 16, 2009; DOI 10.1182/blood-2008-12-195453.
Submitted December 31, 2008
Division of Hematology-Oncology, Department of Medicine, Sylvester Comprehensive Cancer Center, University of Miami, Miami, FL, United States * Corresponding author; email: ilossos{at}med.miami.edu.
Lymphomas of the ocular adnexa are a heterogeneous group of malignancies, comprising approximately 1-2% of non-Hodgkin lymphomas (NHL) and 8% of extranodal lymphomas. The most common subtype, accounting for up to 80% of cases of primary ocular adnexal lymphoma, is marginal zone lymphoma of MALT type. In the recent past, there have been significant advances in our understanding of the clinical characteristics, morphology and phenotype, etiology, pathogenesis, diagnosis, natural history, treatment approaches, outcome and prognostic factors of this disease entity. Novel immunologic and molecular techniques have aided in the distinction between MALT lymphoma and other lymphoproliferative disorders and led to the identification of tissue markers of prognostic significance. Modern imaging modalities provide invaluable tools for accurate staging and treatment planning. Besides radiotherapy and chemotherapy, a variety of new treatment options have emerged in the management of patients with ocular adnexal MALT lymphoma, especially monoclonal antibody therapy and antibiotic therapy against Chlamydia psittaci, which has been associated with the pathogenesis of ocular adnexal lymphomas in some parts of the world. In this review, we are presenting a state of the art summary on ocular adnexal MALT lymphomas.
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