title: EBV-positive extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in the posttransplant setting: a distinct type of posttransplant lymphoproliferative disorder?
abstract: 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 κ positive, 1 case λ 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.
authors.affiliation: Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA.
authors.forename: Sarah E
authors.initials: SE
authors.lastname: Gibson
authors.forename: Steven H
authors.initials: SH
authors.lastname: Swerdlow
authors.forename: Fiona E
authors.initials: FE
authors.lastname: Craig
authors.forename: Urvashi
authors.initials: U
authors.lastname: Surti
authors.forename: James R
authors.initials: JR
authors.lastname: Cook
authors.forename: Michael A
authors.initials: MA
authors.lastname: Nalesnik
authors.forename: Chris
authors.initials: C
authors.lastname: Lowe
authors.forename: Katrina M
authors.initials: KM
authors.lastname: Wood
authors.forename: Chris M
authors.initials: CM
authors.lastname: Bacon
mesh_terms: D000328:Adult; D000368:Aged; D000998:Antiviral Agents / Q000627:therapeutic use; D002648:Child; D003131:Combined Modality Therapy; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology* / Q000821:virology; D005260:Female; D005838:Genotype; D016027:Heart Transplantation / Q000009:adverse effects; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017404:In Situ Hybridization, Fluorescence; D016030:Kidney Transplantation / Q000009:adverse effects; D008206:Lymphatic Diseases / Q000473:pathology / Q000821:virology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000209:etiology / Q000473:pathology* / Q000628:therapy / Q000821:virology; D008297:Male; D016377:Organ Transplantation / Q000009:adverse effects*; D016035:Pancreas Transplantation / Q000009:adverse effects; D011183:Postoperative Complications; D012367:RNA, Viral / Q000235:genetics / Q000302:isolation & purification; D011878:Radiotherapy; D012983:Soft Tissue Neoplasms / Q000209:etiology / Q000473:pathology* / Q000628:therapy / Q000821:virology; D055815:Young Adult
chemical_list: D000998:Antiviral Agents; D007166:Immunosuppressive Agents; D012367:RNA, Viral