Plasma cell neoplasms

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Plasma cell neoplasms arise from plasma cells. They are encountered by anatomical pathologists on occasion.

VL does not tease apart plasma cell myeloma, plasmacytoma (solitary myeloma)[1] and plasma cell neoplasm; the first two of these terms redirect to this article.

General

  • Malignancy derived from the plasma cells.
  • Prognosis: poor.
  • Common primary bone tumour in adults.

Clinical:[2]

  • Bence Jones protein (urine).
  • Abnormal protein electrophoresis (monoclonal gammopathy, dysproteinemia, paraproteinemia).

Note:

  • Plasmacytoma = histology of multiple myeloma; to diagnose multiple myeloma other (non-pathology) criteria are needed.

Multiple myeloma

Features of multiple myeloma (mnemonic CARL):

  • Calcium (in the serum) is elevated.
  • Anemia.
  • Renal failure.
  • Lytic bone lesions.

CRAB (calclium, renal failure, anemia, bony lesions) is another mnemonic.[3]

Microscopic

Features (plasmacytoma):

Images:

DDx:

  • Neuroendocrine carcinoma - nucleus often has a plasmacytoid (plasma cell-like) appearance.


IHC

  • Kappa -- usu. slightly stronger than lambda.
  • Lambda.
  • CD56.[6]
    • Also +ve in NK/T cell lymphomas.
  • CD57.
    • Also +ve in T-cell large granular lymphocytic leukemia.[7].
  • CD138.
  • CD38. (???)

Molecular

  • t(4;14)(p16.3;q32.3) / IGH–MMSET.[8]
    • Associated with poor prognosis.[9]
  • 13q deletion.
    • Worse prognosis.[1]
  • 17q deletion.
    • Worse prognosis.[1]

See also

References

  1. 1.0 1.1 1.2 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. 324. ISBN 978-1416054542.
  2. 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. 323. ISBN 978-1416054542.
  3. "Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group.". Br J Haematol 121 (5): 749-57. Jun 2003. PMID 12780789.
  4. URL: http://www.thefreelibrary.com/Dutcher+bodies+in+chronic+synovitis-a083551789. Accessed on: 4 August 2010.
  5. URL: http://picasaweb.google.com/115272207060951660904/HistiocyteDisorders. Accessed on: 10 August 2011.
  6. URL: http://www.ncbi.nlm.nih.gov/omim/116930. Accessed on: 31 August 2010.
  7. URL: http://www.nature.com/bmt/journal/v33/n1/full/1704298a.html. Accessed on: 31 August 2010.
  8. Chesi, M.; Nardini, E.; Lim, RS.; Smith, KD.; Kuehl, WM.; Bergsagel, PL. (Nov 1998). "The t(4;14) translocation in myeloma dysregulates both FGFR3 and a novel gene, MMSET, resulting in IgH/MMSET hybrid transcripts.". Blood 92 (9): 3025-34. PMID 9787135.
  9. Keats, JJ.; Reiman, T.; Maxwell, CA.; Taylor, BJ.; Larratt, LM.; Mant, MJ.; Belch, AR.; Pilarski, LM. (Feb 2003). "In multiple myeloma, t(4;14)(p16;q32) is an adverse prognostic factor irrespective of FGFR3 expression.". Blood 101 (4): 1520-9. doi:10.1182/blood-2002-06-1675. PMID 12393535.