extranodal marginal zone B-cell lymphoma; low grade B-cell lymphoma of MALT [mucosa associated lymphoid tissue] type, MALToma
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Introduction
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
- gastric MALT lymphoma arises following chronic infection with H pylori
- may arise in association with autoimmune disease
- Sjogren syndrome
- Hashimoto thyroiditis
- lymphoepithelial sialadenitis
Epidemiology
- accounts for 50% of gastric lymphomas
- 7-8% of B cell lymphomas
- generally occurs in older patients, median age 61
Pathology
- tumors are low-grade
- dense infiltrates of B lymphocytes of the mucosal lymphoid tissue
- tumors often remain restricted to mucosa, but may become invasive & life-threatening
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 +
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
Clinical manifestations
- variable presentations
Management
- 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]
- surgical resection with involved field radiation therapy for tumors that do not respond to eradication of H pylori[2]
- chemotherapy as indicated
More general terms
References
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/7741099
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16,17, 18, 19. College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018, 2021.
- ↑ WHO Classification Tumours of Haematopoietic and Lymphoid Tissues. IARC Press 2001.
- ↑ 4.0 4.1 Ferreri AJ, Zucca E. Marginal-zone lymphoma. Crit Rev Oncol Hematol. 2007 Sep;63(3):245-56. Epub 2007 Jun 20. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17583528
- ↑ 5.0 5.1 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: https://www.ncbi.nlm.nih.gov/pubmed/19193705
- ↑ 6.0 6.1 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: https://www.ncbi.nlm.nih.gov/pubmed/20586708
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/10887100
- ↑ 8.0 8.1 Lalya I, Mansouri H Bilateral Lower Palpebral MALT Lymphoma N Engl J Med 2015; 372:363. January 22, 2015 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/25607429 <Internet> http://www.nejm.org/doi/full/10.1056/NEJMicm1400091
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/26989205 Free Article
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/16112347
- ↑ Rossi D, Bertoni F, Zucca E. Marginal-Zone Lymphomas. N Engl J Med 2022; 386:568-581 PMID: https://www.ncbi.nlm.nih.gov/pubmed/35139275 https://www.nejm.org/doi/full/10.1056/NEJMra2102568