{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T23:51:45Z","timestamp":1775778705336,"version":"3.50.1"},"reference-count":45,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2005,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Marginal-zone lymphoma (MZL) includes three subtypes depending on the site of lymphoma involvement: extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT-lymphoma); splenic MZL; and nodal MZL. Beside a common cell-of-origin and similarities concerning a possible chronic antigenic stimulation by microbial pathogens and\/or autoantigens, the clinical presentation is very different with symptoms related to lymphoma location. MALT and splenic MZL present with an indolent disease with good performance status, no B symptoms, and no adverse prognostic factors and are associated with long survival. Patients with nodal MZL present with a more aggressive disease and have a shorter failure-free survival. Clinical and biological prognostic factors identified in reported series are heterogeneous. The optimal treatment has yet to be defined for the three subtypes, and current strategies will be described in this review.<\/jats:p>","DOI":"10.1182\/asheducation-2005.1.307","type":"journal-article","created":{"date-parts":[[2005,11,23]],"date-time":"2005-11-23T01:29:34Z","timestamp":1132709374000},"page":"307-313","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":90,"title":["Clinical Presentation and Management of Marginal Zone Lymphomas"],"prefix":"10.1182","volume":"2005","author":[{"given":"Catherine","family":"Thieblemont","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"234","reference":[{"key":"2019111706094519000_R1","doi-asserted-by":"crossref","unstructured":"Maes B, De Wolf-Peeters C. Marginal zone cell lymphoma\u2014an update on recent advances. Histopathology. 2002;40:117\u2013126.","DOI":"10.1046\/j.1365-2559.2002.01360.x"},{"key":"2019111706094519000_R2","doi-asserted-by":"crossref","unstructured":"Harris NL, Jaffe ES, Stein H, et al. A revised European-American Classification of lymphoid neoplasms. A proposal from the International Lymphoma Study Group. Blood. 1994;84:1361\u20131392.","DOI":"10.1182\/blood.V84.5.1361.1361"},{"key":"2019111706094519000_R3","unstructured":"Jaffe ES, Harris NL, Stein H, Vardiman JW. World Health Organization Classification of Tumours: Pathology and genetics of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2001."},{"key":"2019111706094519000_R4","doi-asserted-by":"crossref","unstructured":"Mulligan SP, Matutes E, Dearden C, Catovsky D. Splenic lymphoma with villous lymphocytes. Natural history and response to therapy in 50 cases. British Journal of Haematology. 1991;78:206\u2013209.","DOI":"10.1111\/j.1365-2141.1991.tb04417.x"},{"key":"2019111706094519000_R5","doi-asserted-by":"crossref","unstructured":"Troussard X, Valensi F, Duchayne E, et al. Splenic lymphoma with villous lymphocytes: clinical presentation, biology and prognostic factors in a series of 100 patients. Groupe Francais d\u2019Hematologie Cellulaire (GFHC). Br J Haematol. 1996;93:731\u2013736.","DOI":"10.1046\/j.1365-2141.1996.d01-1711.x"},{"key":"2019111706094519000_R6","doi-asserted-by":"crossref","unstructured":"Thieblemont C, Felman P, Callet-Bauchu E, et al. Splenic marginal-zone lymphoma: a distinct clinical and pathological entity. Lancet Oncol. 2003;4:95\u2013103.","DOI":"10.1016\/S1470-2045(03)00981-1"},{"key":"2019111706094519000_R7","doi-asserted-by":"crossref","unstructured":"Nathwani B, Anderson J, Armitage J, et al. Marginal zone B-cell lymphoma: A clinical comparison of nodal and mucosa-associated lymphoid tissue types. Non-Hodgkin\u2019s Lymphoma Classification Project. J Clin Oncol. 1999;17:2486\u20132492.","DOI":"10.1200\/JCO.1999.17.8.2486"},{"key":"2019111706094519000_R8","doi-asserted-by":"crossref","unstructured":"Berger F, Felman P, Thieblemont C, et al. Non-MALT marginal zone B-cell lymphomas: a description of clinical presentation and outcome in 124 patients. Blood. 2000;95:1950\u20131956.","DOI":"10.1182\/blood.V95.6.1950"},{"key":"2019111706094519000_R9","doi-asserted-by":"crossref","unstructured":"Farinha P, Gascoyne R. Helicobacter pylori and MALT Lymphoma. Gastroenterology. 2005;128:1579\u20131605.","DOI":"10.1053\/j.gastro.2005.03.083"},{"key":"2019111706094519000_R10","doi-asserted-by":"crossref","unstructured":"Cerroni L, Zochling N, Putz B, Kerl H. Infection by Borrelia burgdorferi and cutaneous B-cell lymphoma. J Cutan Pathol. 1997;24:457\u2013461.","DOI":"10.1111\/j.1600-0560.1997.tb01318.x"},{"key":"2019111706094519000_R11","doi-asserted-by":"crossref","unstructured":"Lecuit M, Abachin E, Martin A, et al. Immunoproliferative small intestinal disease associated with Campylobacter jejuni. N Engl J Med. 2004;350:239\u2013248.","DOI":"10.1056\/NEJMoa031887"},{"key":"2019111706094519000_R12","doi-asserted-by":"crossref","unstructured":"Ferreri A, Guidoboni M, Ponzoni M, et al. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst. 2004;96:586\u2013594.","DOI":"10.1093\/jnci\/djh102"},{"key":"2019111706094519000_R13","doi-asserted-by":"crossref","unstructured":"Zucca E, Conconi A, Pedrinis E, et al. Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood. 2003;101:2489\u20132495.","DOI":"10.1182\/blood-2002-04-1279"},{"key":"2019111706094519000_R14","doi-asserted-by":"crossref","unstructured":"Arcaini L, Paulli M, Boveri E, et al. Splenic and nodal marginal zone lymphomas are indolent disorders at high hepatitis C virus seroprevalence with distinct presenting features but similar morphologic and phenotypic profiles. Cancer. 2004;100:107\u2013115.","DOI":"10.1002\/cncr.11893"},{"key":"2019111706094519000_R15","doi-asserted-by":"crossref","unstructured":"Pinotti G, Zucca E, Roggero E, et al. Clinical features, treatment and outcome in a series of 93 patients with low-grade gastric MALT lymphoma. Leuk Lymphoma. 1997;26:527\u2013537.","DOI":"10.3109\/10428199709050889"},{"key":"2019111706094519000_R16","doi-asserted-by":"crossref","unstructured":"Thieblemont C, Bastion Y, Berger F, et al. Mucosa-associated lymphoid tissue gastrointestinal and nongastrointestinal lymphoma behavior: analsyis of 108 patients. J Clin Oncol. 1997;15:1624\u20131630.","DOI":"10.1200\/JCO.1997.15.4.1624"},{"key":"2019111706094519000_R17","doi-asserted-by":"crossref","unstructured":"Thieblemont C, Berger F, Dumontet C, et al. Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood. 2000;95:802\u2013806d.","DOI":"10.1182\/blood.V95.3.802.003k19_802_806"},{"key":"2019111706094519000_R18","doi-asserted-by":"crossref","unstructured":"Copie-Bergman C, Gaulard P, Lavergne-Slove A, et al. Proposal for a new histological grading system for post-treatment evaluation of gastric MALT lymphoma. Gut. 2003;52:1656.","DOI":"10.1136\/gut.52.11.1656"},{"key":"2019111706094519000_R19","doi-asserted-by":"crossref","unstructured":"Conconi A, Martinelli G, Thieblemont C, et al. Clinical activity of rituximab in extranodal marginal zone B-cell lymphoma of MALT type. Blood. 2003;15:2741\u20132745.","DOI":"10.1182\/blood-2002-11-3496"},{"key":"2019111706094519000_R20","doi-asserted-by":"crossref","unstructured":"Wotherspoon A, Doglioni C, Diss T, et al. Regression of primary low-grade B-cell gastric lymphoma of mucosa associated lymphoid tissue type after eradication of Helicobacter pylori. Lancet. 1993;342:575\u2013577.","DOI":"10.1016\/0140-6736(93)91409-F"},{"key":"2019111706094519000_R21","doi-asserted-by":"crossref","unstructured":"Roggero E, Zucca E, Mainetti C, et al. Eradication of Borrelia burgdorferi infection in primary marginal zone B-cell lymphoma of the skin. Hum Pathol. 2000;31:263\u2013268.","DOI":"10.1016\/S0046-8177(00)80233-6"},{"key":"2019111706094519000_R22","doi-asserted-by":"crossref","unstructured":"Bertoni F, Conconi A, Capella C, et al. Molecular follow-up in gastric mucosa-associated lymphoid tissue lymphomas: early analysis of the LY03 cooperative trial. Blood. 2002;99:2541\u20132544.","DOI":"10.1182\/blood.V99.7.2541"},{"key":"2019111706094519000_R23","doi-asserted-by":"crossref","unstructured":"Tsang R, Gospodarowicz M, Pintilie M, et al. Stage I and II MALT lymphoma: results of treatment with radiotherapy. Int J Radiat Oncol Biol Phys. 2001;50:1258\u20131264.","DOI":"10.1016\/S0360-3016(01)01549-8"},{"key":"2019111706094519000_R24","doi-asserted-by":"crossref","unstructured":"Schechter N, Portlock C, Yahalom J. Treatment of mucosa-asociated lymphoid tissue lymphoma of the stomach with radiation alone. J Clin Oncol. 1998;16:1916\/1921.","DOI":"10.1200\/JCO.1998.16.5.1916"},{"key":"2019111706094519000_R25","doi-asserted-by":"crossref","unstructured":"Hammel P, Haioun C, Chaumette M, et al. Efficacy of single-agent chemotherapy in low-grade B-cell mucosa-associated lymphoid tissue lymphoma with prominent gastric expression. J Clin Oncol. 1995;13:2524\u20132529.","DOI":"10.1200\/JCO.1995.13.10.2524"},{"key":"2019111706094519000_R26","doi-asserted-by":"crossref","unstructured":"Martinelli G, Laszlo D, Ferreri A, et al. Clinical activity of rituximab in gastric marginal zone non-Hodgkin\u2019s lymphoma resistant to or not eligible for anti-Helicobacter pylori therapy. J Clin Oncol. 2005;23:1979\u20131983.","DOI":"10.1200\/JCO.2005.08.128"},{"key":"2019111706094519000_R27","doi-asserted-by":"crossref","unstructured":"Radaszkiewicz T, Dragosics B, Bauer P. Gastrointestinal malignant lymphomas of the mucosa-associated lymphoid tissue: factors relevant to prognosis. Gastroenterology. 1992;102:1628\u20131638.","DOI":"10.1016\/0016-5085(92)91723-H"},{"key":"2019111706094519000_R28","doi-asserted-by":"crossref","unstructured":"Montalban C, Castrillo J, Abraira V, et al. Gastric B-cell mucosa-associated lymphoid tissue (MALT) lymphoma. Clinicopathological study and evaluation of the prognostic factors in 143 patients. Ann Oncol. 1995;6:355\u2013362.","DOI":"10.1093\/oxfordjournals.annonc.a059184"},{"key":"2019111706094519000_R29","doi-asserted-by":"crossref","unstructured":"Liu H, Ruskon-Fourmestraux A, Lavergne-Slove A, et al. Resistance of t(11;18) positive gastric mucosa-associated lymphoid tissue lymphoma to Helicobacter pylori eradication therapy. Lancet. 2001;357:39\u201340.","DOI":"10.1016\/S0140-6736(00)03571-6"},{"key":"2019111706094519000_R30","doi-asserted-by":"crossref","unstructured":"Levy M, Copie-Bergman C, Gameiro C, et al. Prognostic value of translocation t(11;18) in tumoral response of low-grade gastric lymphoma of mucosa-associated lymphoid tissue type to oral chemotherapy. J Clin Oncol. 2005;23:5061\u20135066.","DOI":"10.1200\/JCO.2005.05.660"},{"key":"2019111706094519000_R31","doi-asserted-by":"crossref","unstructured":"Thieblemont C, Felman P, Berger F, et al. Treatment of splenic marginal zone B-cell lymphoma: an analysis of 81 patients. Clin Lymphoma. 2002;3:41\u201347.","DOI":"10.3816\/CLM.2002.n.010"},{"key":"2019111706094519000_R32","doi-asserted-by":"crossref","unstructured":"Talamini R, Montella M, Crovatto M, et al. Non-Hodgkin\u2019s lymphoma and hepatitis C virus: a case-control study from northern and southern Italy. Int J Cancer. 2004;110:380\u2013385.","DOI":"10.1002\/ijc.20137"},{"key":"2019111706094519000_R33","doi-asserted-by":"crossref","unstructured":"Hermine O, Lefrere F, Bronowicki J, et al. Regression of splenic lymphoma with villous lymphocytes after treatment of hepatitis C virus infection. N Engl J Med. 2002;11:89\u201394.","DOI":"10.1056\/NEJMoa013376"},{"key":"2019111706094519000_R34","doi-asserted-by":"crossref","unstructured":"Chacon J, Mollejo M, Munoz E, et al. Splenic marginal zone lymphoma: clinical characteristics and prognostic factors in a series of 60 patients. Blood. 2002;100:1648\u20131654.","DOI":"10.1182\/blood.V100.5.1648.h81702001648_1648_1654"},{"key":"2019111706094519000_R35","unstructured":"Catovsky D, Matutes E. Splenic lymphoma with circulating villous lymphocytes\/splenic marginal-zone lymphoma. Semin Hematol. 1999;36:148\u2013154."},{"key":"2019111706094519000_R36","doi-asserted-by":"crossref","unstructured":"Franco V, Florena A, Stella M, et al. Splenectomy influences bone marrow infiltration in patients with splenic marginal zone cell lymphoma with or without villous lymphocytes. Cancer. 2001;91:294\u2013301.","DOI":"10.1002\/1097-0142(20010115)91:2<294::AID-CNCR1001>3.0.CO;2-W"},{"key":"2019111706094519000_R37","doi-asserted-by":"crossref","unstructured":"Lefrere F, Hermine O, Belanger C, et al. Fludarabine: an effective treatment in patients with splenic lymphoma with villous lymphocytes. Leukemia. 2000;14:573\u2013575.","DOI":"10.1038\/sj.leu.2401710"},{"key":"2019111706094519000_R38","doi-asserted-by":"crossref","unstructured":"Lefrere F, Hermine O, Francois S, et al. Lack of efficacy of 2-chlorodeoxyadenoside in the treatment of splenic lymphoma with villous lymphocytes. Leuk Lymph. 2000;40:113\u2013117.","DOI":"10.3109\/10428190009054887"},{"key":"2019111706094519000_R39","doi-asserted-by":"crossref","unstructured":"Camacho FI, Mollejo M, Mateo MS, et al. Progression to large B-cell lymphoma in splenic marginal zone lymphoma\u2014a description of a series of 12 cases. Am J Surg Pathol. 2001;25:1268\u20131276.","DOI":"10.1097\/00000478-200110000-00007"},{"key":"2019111706094519000_R40","doi-asserted-by":"crossref","unstructured":"Neubauer A, Thiede C, Morgner A, et al. Cure of Helicobacter pylori infection and duration of remission of low-grade gastric mucosa-associated lymphoid tissue lymphoma. J Natl Cancer Inst. 1997;89:1350\u20131355.","DOI":"10.1093\/jnci\/89.18.1350"},{"key":"2019111706094519000_R41","unstructured":"Berger F. The different entities and diagnostic problems. Educational Program of the European Hematology Association meeting. Birmingham. 2000;5."},{"key":"2019111706094519000_R42","doi-asserted-by":"crossref","unstructured":"Koh L, Lim L, Thng C. Retreatment with chimeric CD 20 monoclonal antibody in a patient with nodal marginal zone B-cell lymphoma. Med Oncol. 2000;17:225\u2013228.","DOI":"10.1007\/BF02780533"},{"key":"2019111706094519000_R43","doi-asserted-by":"crossref","unstructured":"Hyjek E, Isaacson P. Primary B cell lymphoma of the thyroid and its relationship to Hashimoto\u2019s thyroiditis. Hum Pathol. 1988;19:1315\u20131326.","DOI":"10.1016\/S0046-8177(88)80287-9"},{"key":"2019111706094519000_R44","doi-asserted-by":"crossref","unstructured":"Hyjek E, Smith W, Isaacson P. Primary B cell lymphoma of salivary glands and its relationship to myoepithial sialadenitis. Hum Pathol. 1988;19:766\u2013776.","DOI":"10.1016\/S0046-8177(88)80259-4"},{"key":"2019111706094519000_R45","doi-asserted-by":"crossref","unstructured":"Zucca E, Bertoni F, Roggero E, Cavalli F. The gastric marginal zone B-cell lymphoma of MALT type. Blood. 2000;96:410\u2013419.","DOI":"10.1182\/blood.V96.2.410"}],"container-title":["Hematology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/ashpublications.org\/hematology\/article-pdf\/2005\/1\/307\/645210\/307.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/ashpublications.org\/hematology\/article-pdf\/2005\/1\/307\/645210\/307.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,11,17]],"date-time":"2019-11-17T11:10:01Z","timestamp":1573989001000},"score":1,"resource":{"primary":{"URL":"https:\/\/ashpublications.org\/hematology\/article\/2005\/1\/307\/19301\/Clinical-Presentation-and-Management-of-Marginal"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,1,1]]},"references-count":45,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2005,1,1]]}},"URL":"https:\/\/doi.org\/10.1182\/asheducation-2005.1.307","relation":{},"ISSN":["1520-4391","1520-4383"],"issn-type":[{"value":"1520-4391","type":"print"},{"value":"1520-4383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,1,1]]}}}