Marginal zone lymphoma

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Marginal zone lymphoma, abbreviated MZL, is a common type of lymphoma composed of small cells.

Marginal zone lymphoma
Diagnosis in short

Marginal zone lymphoma of the kidney. H&E stain.
LM small lymphoid cells +/-plasmacytoid features +/-lymphoepithelial lesion
LM DDx other small cell lymphomas, DLBCL
IHC CD20 +ve, BCL2 +ve, CD21 +ve, CD43 +ve/-ve
Site gastrointestinal tract, lymph nodes
Prevalence moderate
Endoscopy +/-nodularity
Other CD11c +ve (flow cytometry or laser scanning cytometry)

General

Classification

  • Comes in three different flavours:
    1. Extranodal marginal zone lymphoma.
      • If in mucosa-associated lymphoid tissue known as a MALT lymphoma, AKA MALToma.
    2. Splenic marginal zone lymphoma (SMZL).
    3. Nodal marginal zone lymphoma (NMZL).

Microscopic

Features:

  • Small (lymphoid) cells that may be plasma cell-like (plasmacytoid):[2]
    • +/-Clockface nucleus.
    • +/-Eccentric nucleus.
  • +/-"Lymphoepithelial lesion" - gastric crypts invaded by a monomorphous population of lymphocytes.[3]
    • Features:
      1. Cluster of lymphocytes - three cells or more - key feature.
        • Single lymphocytes don't count.
      2. Clearing around the lymphocyte cluster.
    • Not specific for MALT lymphoma, i.e. may be seen in other types of lymphoma.[4]

DDx:

Images

  • MZL - low mag.

  • MZL - intermed. mag.

  • MZL - high mag.

  • MZL - very high mag.

  • MZL - very high mag.

www:

Lymphoepithelial lesion

  • Lymphoepithelial lesion - very high mag. (WC)

  • Lymphoepithelial lesion - intermed. mag. (WC)

IHC

Features:[5]

  • CD20 +ve.
  • BCL2 +ve.
  • CD21 +ve.
  • CD11c +ve (flow cytometry or laser scanning cytometry - only; not available for paraffin).
  • CD43 +ve/-ve.

Others:

  • CD5 -ve.
  • CD10 -ve.
  • CD23 -ve.

Molecular

There are several associated with MALT lymphoma:[6]

  • t(11;18)(q21;q21) / API2‐MALT1[7] - most common translocation in MALT lymphoma.[8]
  • t(14;18)(q32;q21) / IGH‐MALT1.
  • t(1;14)(p22;q32) / IGH‐BCL10.

The MALT1 associated translocations can be assessed with an ISH break apart probe for MALT1.

See also

References

  1. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 326. ISBN 978-1416054542.
  2. ↑ URL: http://surgpathcriteria.stanford.edu/bcell/marginalnodal/printable.html. Accessed on: 6 March 2012.
  3. ↑ Papadaki, L.; Wotherspoon, AC.; Isaacson, PG. (Nov 1992). "The lymphoepithelial lesion of gastric low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT): an ultrastructural study.". Histopathology 21 (5): 415-21. PMID 1452124.
  4. ↑ DB. 6 August 2010.
  5. ↑ Lester, Susan Carole (2005). Manual of Surgical Pathology (2nd ed.). Saunders. pp. 95. ISBN 978-0443066450.
  6. ↑ Bacon CM, Du MQ, Dogan A (April 2007). "Mucosa-associated lymphoid tissue (MALT) lymphoma: a practical guide for pathologists". J. Clin. Pathol. 60 (4): 361–72. doi:10.1136/jcp.2005.031146. PMC 2001121. PMID 16950858. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2001121/.
  7. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 170. ISBN 978-1416054542.
  8. ↑ Streubel, B.; Lamprecht, A.; Dierlamm, J.; Cerroni, L.; Stolte, M.; Ott, G.; Raderer, M.; Chott, A. (Mar 2003). "T(14;18)(q32;q21) involving IGH and MALT1 is a frequent chromosomal aberration in MALT lymphoma.". Blood 101 (6): 2335-9. doi:10.1182/blood-2002-09-2963. PMID 12406890.
  9. ↑ Vitolo, U.; Ferreri, AJ.; Montoto, S. (Jun 2008). "Follicular lymphomas.". Crit Rev Oncol Hematol 66 (3): 248-61. doi:10.1016/j.critrevonc.2008.01.014. PMID 18359244.
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