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EBV-Positive Extranodal Marginal Zone Lymphoma of Mucosa-associated Lymphoid Tissue in the Posttransplant Setting: A Distinct Type of Posttransplant Lymphoproliferative Disorder?

Lookup NU author(s): Dr Christopher Bacon


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The 2008 World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues defines monomorphic posttransplant lymphoproliferative disorders (M-PTLDs) as lymphoid or plasmacytic proliferations that fulfill the criteria for one of the B-cell or T/NK-cell neoplasms recognized in immunocompetent patients. However, indolent B-cell lymphomas, such as extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), are specifically excluded from this category. In this study, we describe the clinicopathologic features of 4 posttransplant lymphoma-like proliferations that were Epstein-Barr virus (EBV) positive, but were otherwise completely typical for a MALT lymphoma. The 4 patients (age, 12 to 71 y) had received solid organ transplants (2 hearts, 1 kidney, 1 kidney/pancreas) at a median of 116 months before presentation, and had been maintained on varying immunosuppressive regimens that included cyclosporine, azathioprine, tacrolimus, and sirolimus. Three of the 4 patients presented with solitary subcutaneous masses, whereas the fourth patient presented with a solitary orbital soft tissue mass. All the 4 cases were morphologically typical for MALT lymphoma, demonstrated plasmacytic differentiation with IgA heavy chain restriction (3 cases kappa positive, 1 case lambda positive), and were diffusely EBV-encoded small RNA positive. Patients were followed for a median of 44.9 months, and all achieved a complete response following various regimens that included reduced immunosuppression with or without antiviral therapy, local surgical excision, rituximab, or local radiation therapy. The uniform EBV positivity and response to immune reconstitution in some cases suggest that EBV-positive MALT lymphomas arising in the posttransplant setting should be included among PTLDs. Whether their distinctive subcutaneous/soft tissue localization and IgA positivity are uniform features will require identification of additional cases.

Publication metadata

Author(s): Gibson SE, Swerdlow SH, Craig FE, Surti U, Cook JR, Nalesnik MA, Lowe C, Wood KM, Bacon CM

Publication type: Article

Publication status: Published

Journal: American Journal of Surgical Pathology

Year: 2011

Volume: 35

Issue: 6

Pages: 807-815

Print publication date: 01/06/2011

ISSN (print): 0147-5185

ISSN (electronic): 1532-0979

Publisher: Lippincott Williams & Wilkins


DOI: 10.1097/PAS.0b013e3182190999


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