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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

  3. Isaacson, P.G., Nathwani, B.N., Piris, M.A. et al. Nodal marginal zone B-cell 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: 161Google Scholar

  4. Isaacson, P.G., Piris, M.A., Catovsky, D. et al. Splenic marginal zone 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: 135–137 Google Scholar

  5. Liliemark, J., Martinsson, U., Cavallin-Stahl, E. et al. Cladribine for untreated or early low-grade non-Hodgkin's lymphoma. Leuk Lymphoma. 1998; 30: 573–581| PubMed

  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

  7. Banks, P.M. Incorporation of immunostaining data in anatomic pathology reports. Am J Surg Pathol. 1992; 16: 808 PubMed

  8. Lennert, K. Malignant lymphomas other than Hodgkin's disease: histology, cytology, ultrastructure, immunology. Springer-Verlag, Berlin; 1978 Google Scholar

  9. Harris, N.L., Jaffe, E.S., Diebold, J. et al. World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee Meeting-Airlie House, Virginia, November 1997 [In Process Citation]. J Clin Oncol. 1999; 17: 3835–3849| PubMed

  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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