Intravascular large B-cell lymphoma

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Intravascular large B-cell lymphoma refers to a rare form of lymphoma that is intravascular, B-cell derived, and does not manifest as a mass.

Intravascular large B-cell lymphoma
Diagnosis in short

Intravascular lymphoma. H&E stain.
LM abundant atypical intravascular lymphoid cells (large ~2-3X size of a mature lymphocyte, nucleolus prominent)
LM DDx T-cell intravascular lymphoma
IHC CD45 +ve, CD20 +ve, CD34 -ve, CD3 -ve
Site blood vessels
Signs non-specific presentation - may include fever, skin lesions
Prevalence rare
Prognosis poor

Several terms have been use to refer this entity, including angiotropic lymphoma, intravascular malignant lymphomatosis and malignant angioendotheliomatosis.

General

  • Rare - est. at one in million.[1]

Clinical:

  • Often a non-specific presentation.[2]
    • +/-Fever.
    • +/-Multiple infarcts.
    • +/-Non-specific skin lesions.

Microscopic

Features:

  • Abundant atypical intravascular lymphoid cells that are:
    • Large (~2-3X size of a mature lymphocyte or RBC).
    • Nucleolus prominent.

Notes:

  • It may be hard to find RBCs in the vessels.
  • Looks sorta like a DLBCL -- but is intravascular.

Images

  • Intravascular lymphoma - high mag. (WC/Nephron)

  • Intravascular lymphoma - very high mag. (WC/Nephron)

www:

IHC

Features:[2]

  • CD20 +ve - key feature.
  • CD79a +ve.[1]
  • CD3 -ve.
  • CD34 -ve.

See also

References

  1. ↑ 1.0 1.1 Raza, M.; Qayyum, S.; Raza, S.; Goorha, S. (May 2012). "Intravascular B-cell lymphoma: an elusive diagnosis.". J Clin Oncol 30 (15): e144-5. doi:10.1200/JCO.2011.38.7720. PMID 22529262.
  2. ↑ 2.0 2.1 2.2 Lapkuviene O, Forchetti D, Roepke JE (October 2001). "Unusual sites of involvement by hematologic malignancies. Case 1. Intravascular large B-cell lymphoma presenting with CNS symptoms". J. Clin. Oncol. 19 (19): 3988–91. PMID 11579120. http://jco.ascopubs.org/content/19/19/3988.full.
  3. ↑ URL: http://path.upmc.edu/cases/case473.html. Accessed on: 21 January 2012.
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