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 CD20 +ve, CD34 -ve, CD3 -ve
Site blood vessels

Signs non-specific presentation - may include fever, skin lesions
Prevalence rare
Prognosis poor
Clin. DDx dependent on presentation, may include stroke

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

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.