The presence of lymphoepithelial lesions is not essential for the diagnosis, and their presence is not pathognomonic, as these can be seen both in some reactive conditions and in other extranodal lymphomas. Dreyling M, Thieblemont C, Gallamini A, et al. A polychemotherapy regimen comprising chlorambucil/mitoxantrone/prednisone has been reported to be effective and well tolerated in MALT lymphoma, irrespective of localization. Overall, doxycycline treatment has been studied in about 120 patients with ocular adnexa MALT lymphoma (Table 3), with responses seen in about half of these. There is no clear consensus for the treatment of patients with gastric MALT lymphoma who require further therapy beyond H pylori eradication or who have extensive disease. 25. 1997;89:3909-18. Role of fluorine-18 fluoro-deoxyglucose positron emission tomography scan in the evaluation and follow-up of patients with low-grade lymphomas. Isaacson PG, Piris MA, Berger F, et al. [72,77,81] This contrasts with results in the better-studied gastric MALT lymphoma (where H pylori–negative patients are generally unresponsive to antibiotic treatment) and might suggest that other doxycycline-sensitive microorganisms may be linked with the lymphoma. 22. If left untreated, the lymphoma can undergo a histologic transformation to a diffuse large B-cell lymphoma. Multiple protein domains mediate interaction between Bcl10 and MALT1. 50. 2008;71:1425-35. The diagnosis of MALT lymphoma should be made in accordance with the current World Health Organization (WHO) classification, and it is recommended that the diagnosis be confirmed by an expert hematopathologist. Evidence supporting a pathogenetic association between this bacterial agent and the development of MALT lymphoma of the ocular adnexa includes the identification of chlamydial antigens in tumor tissue by immunohistochemistry, detection of bacterial DNA in tumor biopsy specimens, visualization of the bacteria within tumor-infiltrating macrophages by electron microscopy, and the isolation of the bacteria from conjunctival swabs and the peripheral blood of patients. Int J Radiat Oncol Biol Phys. Thieblemont C, Bastion Y, Berger F, et al. MALT lymphomas are categorized as indolent or low-grade lymphomas. Histologic transformation in marginal zone lymphomas. Curr Hematol Malig Rep. 2011;6:216-21. Cancer. J Clin Oncol. Because of the risk of occult disseminated disease, extensive work-up procedures are recommended in all MALT lymphomas, irrespective of their presentation site,[28] and should comprise a history and physical examination (including lymph node regions, eye and ear, nose and throat, liver and spleen clinical evaluations); complete blood cell counts and basic biochemical studies, including evaluation of renal and liver function; LDH and β2-microglobulin levels; serum protein immunofixation; human immunodeficiency virus (HIV), HCV, and hepatitis B virus (HBV) serologies; CT scan of the chest, abdomen, and pelvis; and bone marrow aspirate and biopsy.