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extranodal marginal zone B-cell lymphoma; low grade B-cell lymphoma of MALT [mucosa associated lymphoid tissue] type, MALToma

Mucosa associated lymphoid tissue (MALT). Classification: - working formulation category: small lymphocytic lymphoma - primary cutaneous marginal cell lymphoma - associated with Borrelia burdorferi infection in Europe - gastric marginal zone lymphoma - associated with Helicobacter pylori Etiology: 1) gastric MALT lymphoma arises following chronic infection with H pylori 2) may arise in association with autoimmune disease - Sjogren syndrome - Hashimoto thyroiditis - lymphoepithelial sialadenitis Epidemiology: 1) accounts for 50% of gastric lymphomas 2) 7-8% of B cell lymphomas 3) generally occurs in older patients, median age 61 Pathology: 1) tumors are low-grade 2) dense infiltrates of B lymphocytes of the mucosal lymphoid tissue 2) tumors often remain restricted to mucosa, but may become invasive & life-threatening Genetics: - t(11;18)(q21;q21) involving MALT1 & BIRC3 genes - t(1;14)(p22;q32) involving BCL10 with IgH - t(1;18) involving API2 [2] - mutations associated with BCL10 Immunophenotype: - IgM typically, less often IgA or IgG - light chain restriction - CD5 - - CD11c +/- weak - CD20 + - CD21 +, CD35 + (marginal zone cell associated antigens) - CD23 - - CD43 +/- - CD79a + Clinical manifestations: - variable presentations - gastric lymphomas - isolated bilateral lower palpebral MALT lymphoma described [8] - unilateral pulmonary [2] Management: 1) eradication of H pylori (if present) is initial treatment of MALT lymphoma [2] - may result in regression & even disappearance of MALT lymphomas [2,5,6] - duration of H pylori treatment 6-8 weeks to months guided by treatment response assessed by repeat upper endoscopy [2] - only effective for gastric MALT lymphoma [2] 2) surgical resection with involved field radiation therapy for tumors that do not respond to eradication of H pylori [2] 3) chemotherapy as indicated a) rituximab (Rituxan) anti-CD20 [2,4] b) alkylating agents

Interactions

disease interactions

General

marginal zone B-cell lymphoma small lymphocytic lymphoma

References

  1. Chan JK, Banks PM, Cleary ML, Delsol G, De Wolf-Peeters C, Falini B, Gatter KC, Grogan TM, Harris NL, Isaacson PG, et al. A revised European-American classification of lymphoid neoplasms proposed by the International Lymphoma Study Group. A summary version. Am J Clin Pathol. 1995 May;103(5):543-60. PMID: 7741099
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16,17, 18, 19. College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018, 2021.
  3. WHO Classification Tumours of Haematopoietic and Lymphoid Tissues. IARC Press 2001.
  4. Ferreri AJ, Zucca E. Marginal-zone lymphoma. Crit Rev Oncol Hematol. 2007 Sep;63(3):245-56. Epub 2007 Jun 20. Review. PMID: 17583528
  5. Stathis A et al. Long-term outcome following Helicobacter pylori eradication in a retrospective study of 105 patients with localized gastric marginal zone B-cell lymphoma of MALT type. Ann Oncol 2009 Jun; 20:1086. PMID: 19193705
  6. Stathis A, Bertoni F, Zucca E. Treatment of gastric marginal zone lymphoma of MALT type. Expert Opin Pharmacother. 2010 Sep;11(13):2141-52 PMID: 20586708
  7. Zucca E, Bertoni F, Roggero E, Cavalli F. The gastric marginal zone B-cell lymphoma of MALT type. Blood. 2000 Jul 15;96(2):410-9. PMID: 10887100
  8. Lalya I, Mansouri H Bilateral Lower Palpebral MALT Lymphoma N Engl J Med 2015; 372:363. January 22, 2015 PMID: 25607429 http://www.nejm.org/doi/full/10.1056/NEJMicm1400091
  9. Zucca E, Bertoni F. The spectrum of MALT lymphoma at different sites: biological and therapeutic relevance. Blood. 2016 Apr 28;127(17):2082-92. Review. PMID: 26989205 Free Article
  10. Wenzel J et al, The role of cytotoxic skin-homing CD8+ lymphocytes in cutaneous T-cell lymphoma and pityriasis lichenoides J Am Acad Dermatol 2005; 53:422 PMID: 16112347
  11. Rossi D, Bertoni F, Zucca E. Marginal-Zone Lymphomas. N Engl J Med 2022; 386:568-581 PMID: 35139275 https://www.nejm.org/doi/full/10.1056/NEJMra2102568