Small cell lymphomas

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The small cell lymphomas are a collection of commonly seen lymphomas that have a near-identical histomorphologic appearance.

The group includes:

  1. Small lymphocytic lymphoma/chronic lymphocytic leukemia.
  2. Follicular lymphoma.
  3. Mantle cell lymphoma.
  4. Marginal zone lymphoma (includes MALT lymphoma).
  5. Hairy cell leukemia.
  6. Immunoproliferative small intestinal disease (IPSID).[1]

Table of B-cell lymphoma

Small cell lymphomas:

Name Location Size of cells IHC Translocations Clinical Other
Follicular lymphoma Follicle Small, centrocytes, centroblasts CD10+, BCL6+[2] t(14;18)(q32;q21) IGH/BCL2[3] may transform into DLBCL very common
Mantle cell lymphoma mantle zone small CD5+, CD23-, CD43+, cyclin D1+[2] t(11;14)(q13;q32) BCL1/IGH[4] (also IGH/BCL1[5]) aggressive, poor prognosis[6] DDx: Castleman disease
Marginal zone lymphoma (includes MALT) marginal zone, spleen, GI tract small CD21+, CD11c+, CD5-, CD23-[2] t(11;18)(q21;q21) / API2‐MALT1, t(14;18)(q32;q21) / IGH‐MALT1, t(1;14)(p22;q32) / IGH‐BCL10[7] classical GI lymphoma subtypes: extranodal marginal zone lymphoma (AKA MALT lymphoma), SMZL, nodal marginal zone lymphoma
Precursor B cell lymphoblastic lymphoma/leukemia location ? small CD10+, CD5-, TdT+, CD99+[2] t(9;22), others good prognosis (?) other ?
B cell small lymphocytic lymphoma /
chronic lymphocytic leukemia 		location ? small CD5+, CD23+, CD43+, cyclin D1- trisomy 12; deletions of 11q, 13q, 17p[8] good prognosis / indolent course other ?

Medium and large cell lymphomas:

Name Location Size of cells IHC Translocations Clinical Other
Burkitt's lymphoma follicle large cells CD10, BCL6 t(8;14) (q24;q32) rapid growth "starry sky"
Diffuse large B cell lymphoma follicle (?) large 4-5X of lymphocyte MIB1 >40% none/like follicular l. poor prognosis common among lymphomas

Follicular lymphoma

  • Abbreviated FL.
Main article: Follicular lymphoma

Mantle cell lymphoma

  • Abbreviated MCL.
Main article: Mantle cell lymphoma

Marginal zone lymphoma

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):[10]
    • +/-Clockface nucleus.
    • +/-Eccentric nucleus.
  • +/-"Lymphoepithelial lesion" - gastric crypts invaded by a monomorphous population of lymphocytes.[11]
    • 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.[12]

Images

  • Lymphoepithelial lesion - very high mag. (WC)

  • Lymphoepithelial lesion - intermed. mag. (WC)

www:

IHC

Features:[13]

  • 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:[7]

  • t(11;18)(q21;q21) / API2‐MALT1[14] - most common translocation in MALT lymphoma.[15]
  • 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.

Hairy cell leukemia

  • Abbreviated HCL.

General

  • Name comes from appearance on blood smear - cell hairy.
  • Do to the biology, dry taps are common.[17]

Clinical:[18]

  • Pancytopenia.
  • Splenic enlargement.
  • No lymphadenopathy.
  • Good prognosis (with treatment), though (likely) not curable.

Gross

Features:[9]

  • Huge beefy red spleen.
    • Red as white pulp obliterated.

Microscopic

Features:[19]

  • Small cells (10-20 micrometers) with "Fried egg"-like appearance:
    • Well-demarcated fuzzy cell borders,
    • Clear/whispy cytoplasm and,
    • Central round nucleus.
      • Peri-nuclear clearing ("water-clear rim"[20]) -- key feature.

DDx:

Images

  • HCL - blood film. (WC)

  • HCL - high mag. (WC)

  • HCL - very high mag. (WC)

www:

IHC

Features:[21]

  • CD20 +ve, CD25 +ve, CD103 +ve.
  • CD5 -ve.

Flow cytometry:

  • CD19 +ve, CD11c +ve, FMC7 +ve.

B cell small lymphocytic lymphoma/chronic lymphocytic leukemia

Main article: B cell small lymphocytic lymphoma/chronic lymphocytic leukemia

Precursor B-cell lymphoblastic lymphoma/leukemia

General

  • Good prognosis.
  • Paediatric - usu. <6 years old.

Microscopic

Features:[22]

  • High mitotic rate.
  • "Starry sky" pattern.
  • Small nucleoli.

IHC

Features:[2]

  • CD10 +ve, TdT +ve, CD99 +ve.
  • CD5 -ve.

Molecular

Subclassification based on molecular abnormalities (translocations, rearrangements):[23]

  • t(9;22) / BCR-ABL.
  • t(1;19) / E2A-PBX1.
  • t(12;21) / ETV-CBFalpha.
  • MLL rearrangement.

Precursor T-cell lymphoblastic lymphoma/leukemia

General

  • Prognosis poor. (???)

Microscopic

Features:

  • Small lymphoid cells. (???)

IHC

Features:[24]

  • TdT +ve, CD34 +ve, CD99 +ve, CD1a +ve/-ve.
  • TIA1 -ve.

See also

References

  1. ↑ Al-Saleem T, Al-Mondhiry H (March 2005). "Immunoproliferative small intestinal disease (IPSID): a model for mature B-cell neoplasms". Blood 105 (6): 2274–80. doi:10.1182/blood-2004-07-2755. PMID 15542584. http://bloodjournal.hematologylibrary.org/cgi/content/long/105/6/2274.>
  2. ↑ 2.0 2.1 2.2 2.3 2.4 Lester, Susan Carole (2005). Manual of Surgical Pathology (2nd ed.). Saunders. pp. 95. ISBN 978-0443066450.
  3. ↑ Yanai, S.; Nakamura, S.; Takeshita, M.; Fujita, K.; Hirahashi, M.; Kawasaki, K.; Kurahara, K.; Sakai, Y. et al. (Dec 2010). "Translocation t(14;18)/IGH-BCL2 in gastrointestinal follicular lymphoma: correlation with clinicopathologic features in 48 patients.". Cancer. doi:10.1002/cncr.25811. PMID 21192062.
  4. ↑ URL: http://atlasgeneticsoncology.org/Anomalies/t1114ID2021.html. Accessed on: 10 August 2010.
  5. ↑ URL: http://www.wipo.int/patentscope/search/en/WO2010059499. Accessed on: 26 May 2011.
  6. ↑ Hankin, RC.; Hunter, SV. (Dec 1999). "Mantle cell lymphoma.". Arch Pathol Lab Med 123 (12): 1182-8. doi:10.1043/0003-9985(1999)1231182:MCL2.0.CO;2. PMID 10583923.
  7. ↑ 7.0 7.1 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/.
  8. ↑ 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. 318. ISBN 978-1416054542.
  9. ↑ 9.0 9.1 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.
  10. ↑ URL: http://surgpathcriteria.stanford.edu/bcell/marginalnodal/printable.html. Accessed on: 6 March 2012.
  11. ↑ 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.
  12. ↑ DB. 6 August 2010.
  13. ↑ Lester, Susan Carole (2005). Manual of Surgical Pathology (2nd ed.). Saunders. pp. 95. ISBN 978-0443066450.
  14. ↑ 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.
  15. ↑ 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.
  16. ↑ 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.
  17. ↑ Galani, KS.; Subramanian, PG.; Gadage, VS.; Rahman, K.; Ashok Kumar, MS.; Shinde, S.; Mahadik, S.; Ansari, R. et al. "Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital.". Indian J Pathol Microbiol 55 (1): 61-5. doi:10.4103/0377-4929.94858. PMID 22499303.
  18. ↑ URL: http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022. Accessed on: 20 August 2010.
  19. ↑ URL: http://emedicine.medscape.com/article/200580-diagnosis. Accessed on: 18 August 2010.
  20. ↑ URL: http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022. Accessed on: 20 August 2010.
  21. ↑ URL: http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029. Accessed on: 20 August 2010.
  22. ↑ DG. 17 August 2010.
  23. ↑ Randolph TR (2004). "Advances in acute lymphoblastic leukemia". Clin Lab Sci 17 (4): 235–45. PMID 15559730. http://findarticles.com/p/articles/mi_qa3890/is_200410/ai_n9429273/pg_2.
  24. ↑ Lester, Susan Carole (2005). Manual of Surgical Pathology (2nd ed.). Saunders. pp. 97. ISBN 978-0443066450.
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