Extranodal Marginal Zone B Cell Lymphoma

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Contents 1 Introduction 2 Classification 3 Etiology 4 Epidemiology 5 Pathology 6 Immunophenotype 7 Genetics 8 Complications 9 Management 10 More General Terms 11 References

Introduction

• Mucosa associated lymphoid tissue (MALT).

Classification

• working formulation category: small lymphocytic lymphoma

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
• 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

Complications

• 
  
• disease interaction(s) of cancer with Alzheimer's disease

Management

• surgical resection
• chemotherapy

• alkylating agents
• rituxin (anti CD20) [4]

• eradication of H pylori may result in regression & even disappearance of MALT lymphomas [5]

More General Terms

• marginal zone B-cell 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: [1]
2. Medical Knowledge Self Assessment Program (MKSAP) 11, 15, American College of Physicians, Philadelphia 1998, 2009
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: [2]
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: [3]

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