Marginal zone lymphomas (MZL)

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Marginal zone lymphomas (MZL) are indolent B-cell lymphomas that represent less than 20% of all lymphomas.

When marginal zone lymphomas involve the lymph nodes they are called "nodal marginal zone B-cell lymphomas" (NMZLs) whereas when they involve places outside the lymph nodes (gastrointestinal tract, thyroid, breast, or skin) they are called "mucosa-associated lymphatic tissue lymphomas" (MALT lymphomas or MALTomas).

The "splenic marginal zone lymphomas" (SMZLs) are considered a third separate subtype. light zone surrounding splenic follicles which contains post follicular memory B cells derived after stimulation of recirculating cells from T cell dependent antigen

Clonal rearrangements of the immunoglobulin genes (heavy and light chains) . The deletion 7q21-32 in 40% of SMZL patients, and translocations of the CDK6 gene located at 7q21.             

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Immunophenotype

 Positive for pan-B markers CD20+, CD19+, BCL2+/- variable, sIgM+ bright-,cyIg + (40% of the cells) and negative for  CD10-,  CD23-, CD11c-, CD3-, CD5- , sIgD-

 The marker distinguished MZL from CLL/SLL: CD5+, CD23+, follicular lymphoma : CD10+, mantle cell lymphoma : CD5+

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References

1. Harris, N.L., Jaffe, E.S., Stein, H. et al. A revised European–American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group [see comments]. Blood. 1994; 84: 1361–1392| PubMed

2. Isaacson, P.G., Muller-Hermelink, H.K., Piris, M.A. et al. Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). in: E.S. Jaffe, N.L. Harris, H. Stein, (Eds.) World Health Organization classification of tumours. Pathology and genetics of tumours of haematopoietic and lymphoid tissues. IARC Press, Lyon; 2001: 157–160 Google Scholar

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6. Tierens, A., Delabie, J., Pittaluga, S. et al. Mutation analysis of the rearranged immunoglobulin heavy chain genes of marginal zone cell lymphomas indicates an origin from different marginal zone B lymphocyte subsets. Blood. 1998; 91: 2381–2386| PubMed

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10. Wotherspoon, A.C., Doglioni, C., de Boni, M. et al. Antibiotic treatment for low-grade gastric MALT lymphoma. ([Letter, comment])Lancet. 1994; 343: 1503| PubMed

11. Wotherspoon, A.C. Gastric MALT lymphoma and Helicobacter pylori. Yale J Biol Med. 1996; 69: 61–6| PubMed

12. Hussell, T., Isaacson, P.G., Crabtree, J.E., and Spencer, J. The response of cells from low-grade B-cell gastric lymphomas of mucosa-associated lymphoid tissue to Helicobacter pylori [see comments]. Lancet. 1993; 342: 571–574| PubMed

13. Parsonnet, J., Hansen, S., Rodriguez, L. et al. Helicobacter pylori infection and gastric lymphoma [see comments]. N Engl J Med. 1994; 330: 1267–1271| PubMed

14. Wotherspoon, A.C., Doglioni, C., Diss, T.C. et al. Regression of low grade B-cell gastric lymphoma of MALT type following eradication of Helicobacter pylori. ([Meeting abstract])J Pathol. 1993; 170: 335A| PubMed

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