Difference between revisions of "Intravascular large B-cell lymphoma"

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#redirect [[Lymphoma#Intravascular large B-cell lymphoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Intravascular lymphoma - very high mag.jpg
| Width      =
| Caption    = Intravascular lymphoma. [[H&E stain]].
| Synonyms  =
| Micro      = abundant atypical intravascular lymphoid cells (large ~2-3X size of a mature lymphocyte, nucleolus prominent)
| Subtypes  =
| LMDDx      = T-cell intravascular lymphoma
| Stains    =
| IHC        = CD20 +ve, CD34 -ve, CD3 -ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[blood vessels]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      = non-specific presentation - may include fever, skin lesions
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    = dependent on presentation, may include [[stroke]]
| Tx        =
}}
'''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.


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.<ref name=pmid22529262>{{Cite journal  | last1 = Raza | first1 = M. | last2 = Qayyum | first2 = S. | last3 = Raza | first3 = S. | last4 = Goorha | first4 = S. | title = Intravascular B-cell lymphoma: an elusive diagnosis. | journal = J Clin Oncol | volume = 30 | issue = 15 | pages = e144-5 | month = May | year = 2012 | doi = 10.1200/JCO.2011.38.7720 | PMID = 22529262 }}</ref>
Clinical:
*Often a non-specific presentation.<ref name=pmid11579120>{{cite journal |author=Lapkuviene O, Forchetti D, Roepke JE |title=Unusual sites of involvement by hematologic malignancies. Case 1. Intravascular large B-cell lymphoma presenting with CNS symptoms |journal=J. Clin. Oncol. |volume=19 |issue=19 |pages=3988–91 |year=2001 |month=October |pmid=11579120 |doi= |url=http://jco.ascopubs.org/content/19/19/3988.full}}</ref>
**+/-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===
<gallery>
Image:Intravascular_lymphoma_-_intermed_mag.jpg | Intravascular lymphoma - intermed. mag. (WC/Nephron)
Image:Intravascular_lymphoma_-_high_mag.jpg | Intravascular lymphoma - high mag. (WC/Nephron)
Image:Intravascular_lymphoma_-_very_high_mag.jpg | Intravascular lymphoma - very high mag. (WC/Nephron)
</gallery>
www:
*[http://jco.ascopubs.org/content/19/19/3988/F1.expansion.html ILBCL (ascopubs.org)].<ref name=pmid11579120/>
*[http://theoncologist.alphamedpress.org/cgi/content/full/11/8/923/F1 ILBCL in bone marrow (alphamedpress.org)].
*[http://annonc.oxfordjournals.org/content/13/9/1503/F1.expansion.html ILBCL - various images (oxfordjournals.org)].
*[http://path.upmc.edu/cases/case473/images/fig01a.jpg ILBCL (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case473.html http://path.upmc.edu/cases/case473.html]. Accessed on: 21 January 2012.</ref>
*[http://path.upmc.edu/cases/case697.html ILBCL - several images (upmc.edu)].
==IHC==
Features:<ref name=pmid11579120/>
*CD20 +ve - '''key feature'''.
*CD79a +ve.<ref name=pmid22529262/>
*CD3 -ve.
*CD34 -ve.
==See also==
*[[Intravascular lymphoma]].
*[[Extramedullary leukemia]].
*[[Lymphoma]].
==References==
{{Reflist|2}}
[[Category:Lymphoma]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]

Latest revision as of 06:53, 9 November 2014

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
Template:Px
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

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

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

  • Intravascular lymphoma - very high mag.jpg

    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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