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Nongastric Marginal-Zone B-Cell MALT Lymphoma: Prognostic Value of Disease Dissemination

^a Division of Hematology and ^c Department of Pathology, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy; ^b Division of Hematology, Ospedali Riuniti, Bergamo, Italy; ^d Division of Hematology, Niguarda Ca’ Granda Hospital, Milano, Italy; ^e Department of Hematology, and ^f Azienda Ospedaliera S. Andrea, University "La Sapienza," Roma, Italy; ^g Division of Hematology, Azienda Ospedaliera S. Giovanni Battista, Torino, Italy; ^h Division of Hematology, Policlinico "S. Maria alle Scotte", University of Siena, Siena, Italy; ⁱ Department of Hematology, S. Bortolo Hospital, Vicenza, Italy; ^j Department of Hematology, Catholic University Medical School, Rome, Italy; ^k Division of Hematology, University of Udine, Udine, Italy

Key Words. MALT lymphoma • Prognosis • Marginal zone • Dissemination

Correspondence: Luca Arcaini, M.D., Division of Hematology, IRCCS Policlinico San Matteo, University of Pavia, Viale Golgi 19, 27100 Pavia, Italy. Telephone: 39-0382-503595; Fax: 39-0382-502250; e-mail: luca.arcaini{at}unipv.it

The aim of this study is to describe the clinical features and define the prognostic significance of disease dissemination in a large series of nongastric marginal-zone B-cell mucosa-associated lymphoid tissue (MALT) lymphomas. We studied 208 patients with nongastric marginal-zone B-cell MALT lymphoma diagnosed and treated from 1991 to 2004. Ninety percent of the patients had a single site of MALT involvement—skin (26%), salivary glands (18%), orbit (14%), Waldeyer’s ring (13%)—and 39% and 28% had nodal involvement and bone marrow involvement, respectively. After a median follow-up of 2.7 years, the median event-free survival (EFS) time was 2.4 years, and the median overall survival (OS) time was not reached. On univariate analysis, the features significantly associated with longer EFS and OS times were the following: single MALT site involvement (OS), localized disease (EFS and OS), no nodal disease (EFS and OS), skin and orbit lymphoma (OS), and stage IV disease without bone marrow involvement (OS). On multivariate analysis, both bone marrow and nodal involvement were associated with shorter OS. This study describes the clinical features and the natural history of nongastric marginal-zone lymphomas and highlights that the dissemination to lymph nodes and bone marrow is associated with a poorer outcome.

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