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

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{{ 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.
'''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.


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==General==
==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:
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>  
*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>  
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===Images===
===Images===
<gallery>
<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_-_high_mag.jpg | Intravascular lymphoma - high mag. (WC/Nephron)
Image:Intravascular_lymphoma_-_very_high_mag.jpg | Intravascular lymphoma - very high mag. (WC/Nephron)
Image:Intravascular_lymphoma_-_very_high_mag.jpg | Intravascular lymphoma - very high mag. (WC/Nephron)
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Features:<ref name=pmid11579120/>
Features:<ref name=pmid11579120/>
*CD20 +ve - '''key feature'''.
*CD20 +ve - '''key feature'''.
*CD79a +ve.<ref name=pmid22529262/>
*CD3 -ve.
*CD3 -ve.
*CD34 -ve.
*CD34 -ve.
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==See also==
==See also==
*[[Intravascular lymphoma]].
*[[Intravascular lymphoma]].
*[[Extramedullary leukemia]].
*[[Lymphoma]].
*[[Lymphoma]].


Latest revision as of 06:53, 9 November 2014

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

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.[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. (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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