Abstract
Mucosa-associated lymphoid tissue (MALT) lymphoma is a low-grade B-cell lymphoma and is usually slow to disseminate. The t(11;18)(q21;q21) translocation has been identified in a subset of MALT lymphoma cases, and API2 and MLT1/MALT1 genes have been implicated in this translocation. However, the clinicopathologic features of t(11;18)-bearing MALT lymphoma have not been fully elucidated, and the optimal therapy for patients with disseminating disease is unknown. We report an outstanding case of MALT lymphoma that showed massive pulmonary infiltration and leukemic transformation during a prolonged course of more than 16 years. Reverse transcriptase-polymerase chain reaction and dual-color fluorescence in situ hybridization analyses confirmed the existence of the API2/MLT1 fusion gene in the lymphoma cells. Peripheral blood lymphoma cells disappeared after glucocorticoid therapy. Although the pulmonary lymphoma was slowly progressive and caused severe hypoxemia despite the continuation of glucocorticoid therapy, treatment with cladribine induced a marked reduction of pulmonary lymphomatous lesions and an improvement of the hypoxemia. These findings show the unique clinical features of this particular indolent B-cell lymphoma with t(11;18) translocation and suggest the potential therapeutic usefulness of glucocorticoid and cladribine.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Non-Hodgkin’s Lymphoma Classification Project. A clinical evalu- ation of the International Lymphoma Study Group classification of non-Hodgkin’s lymphoma. Blood. 1997;89:3909–3918.
Thieblemont C, Bastion Y, Berger F, et al. Mucosa-associated lym- phoid tissue gastrointestinal and nongastrointestinal lymphoma behavior: analysis of 108 patients. J Clin Oncol. 1997;15:1624–1630.
Isaacson PG. The MALT lymphoma concept updated. Ann Oncol. 1995;6:319–320.
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;95:802–806.
Auer IA, Gascoyne RD, Connors JM, et al. t(11;18)(q21;q21) is the most common translocation in MALT lymphomas. Ann Oncol. 1997;8:979–985.
Ott G, Katzenberger T, Greiner A, et al. The t(11;18)(q21;q21) chromosome translocation is a frequent and specific aberration in low-grade but not high-grade malignant non-Hodgkin’s lymphomas of the mucosa-associated lymphoid tissue (MALT-) type. Cancer Res. 1997;57:3944–3948.
Dyer MJS. The pathogenetic role of oncogenes deregulated by chromosomal translocation in B-cell malignancies. Int J Hematol. 2003;77:315–320.
Kobayashi Y, Nakata M, Maekawa M, et al. Detection of t(11;18) in MALT-type lymphoma with dual-color fluorescence in situ hybridization and reverse transcriptase-polymerase chain reaction analysis. Diagn Mol Pathol. 2001;10:207–213.
Saven A, Emanuele S, Kosty M, Koziol J, Ellison D, Piro L. 2-Chlorodeoxyadenosine activity in patients with untreated, indolent non-Hodgkin’s lymphoma. Blood. 1995;86:1710–1716.
Dierlamm J, Baens M, Wlodarska I, et al. The apoptosis inhibitor gene API2 and a novel 18q gene, MLT, are recurrently rearranged in the t(11;18)(q21;q21) associated with mucosa-associated lymphoid tissue lymphomas. Blood. 1999;93:3601–3609.
Ye H, Liu H, Attygalle A, et al. Variable frequencies of t(11;18) (q21;q21) in MALT lymphomas of different sites: significant association with CagA strains of H pylori in gastric MALT lymphoma. Blood. 2003;102:1012–1028.
Okabe M, Inagaki H, Oshima K, et al. API2-MALT1 fusion defines a distinctive clinicopathologic subtype in pulmonary extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Am J Pathol. 2003;162:1113–1122.
Zinzani PL, Magagnoli M, Galieni P, et al. Nongastrointestinal low- grade mucosa-associated lymphoid tissue lymphoma: analysis of 75 patients. J Clin Oncol. 1999;17:1254–1258.
Zucca E, Roggero E, Pileri S. B-cell lymphoma of MALT type: a review with special emphasis on diagnostic and management problems of low-grade gastric tumours. Br J Haematol. 1998;100:3–14.
Zucca M, Conconi A, Pedrinis E, et al. Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood. 2003;101:2489–2495.
Asatiani E, Cohen P, Ozdemirli M, et al. Monoclonal gammopathy in extranodal subtypes of marginal zone lymphoma (ENMZL) cor- relates with advanced disease with involvement of bone marrow [abstract]. Blood. 2003;102. Abstract 2337.
Conconi A, Martinelli G, Thieblemont A, et al. Clinical activity of rituximab in extranodal marginal zone B-cell lymphoma of MALT type. Blood. 2003;102:2741–2745.
Jaeger G, Neumeister P, Bezinschek R, et al. Treatment of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type with cladribine: a phase II study. J Clin Oncol. 2002;20:3872–3877.
Tobinai K, Kobayashi Y, Morishima Y, et al. Prolonged cytopenia and myelodysplastic syndrome after cladribine treatment in relapsed patients with indolent non-Hodgkin’s lymphoma: results of Japanese phase II study [abstract]. Proc Am Soc Clin Oncol. 2001;20:228b. Abstract 2664.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Kusumoto, S., Kobayashi, Y., Tanimoto, T.E. et al. t(11;18)-Bearing Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma Responding to Cladribine. Int J Hematol 80, 70–74 (2004). https://doi.org/10.1532/IJH97.03170
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1532/IJH97.03170