Marginal zone lymphoma

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)

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

General

Arise in the context chronic infections, e.g. Sjögren disease (salivary gland), Hashimoto thyroiditis (thyroid gland), Helicobacter pylori gastritis (stomach).^[1]

Classification

Comes in three different flavours:
1. Extranodal marginal zone lymphoma.
  - If in mucosa-associated lymphoid tissue known as a MALT lymphoma, AKA MALToma. There is also a counterpart in the lung that arises from bronchus-associated lymphoid tissue. These are known as BALTomas.
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:

Other small cell lymphomas.
DLBCL - should be a distinctive region at low power.^{[citation needed]}

Images

MZL - low mag. (WC)
MZL - intermed. mag. (WC)
MZL - high mag. (WC)
MZL - very high mag. (WC)
MZL - very high mag. (WC)

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.
- Typically positive in mantle cell lymphoma.

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.
- Should not be confused with t(14;18) seen in follicular lymphoma between IGH-BCL2.^[9]
t(1;14)(p22;q32) / IGH‐BCL10.

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

References

↑ 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.
↑ URL: http://surgpathcriteria.stanford.edu/bcell/marginalnodal/printable.html. Accessed on: 6 March 2012.
↑ 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.
↑ DB. 6 August 2010.
↑ Lester, Susan Carole (2005). Manual of Surgical Pathology (2nd ed.). Saunders. pp. 95. ISBN 978-0443066450.
↑ 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/.
↑ 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.
↑ 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.
↑ 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.

[Ref_PCPBoD8_326-1] 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.

[pmid1452124-3] 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.

[pmid16950858-6] 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/.

[Ref_PCPBoD8_170-7] 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.

[pmid12406890-8] 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.

[pmid18359244-9] 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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Marginal zone lymphoma

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