Difference between revisions of "Burkitt lymphoma"

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| Subtypes  = endemic, non-endemic, immunodeficiency associated
| Subtypes  = endemic, non-endemic, immunodeficiency associated
| LMDDx      = [[DLBCL]], intermediate DLBCL/BL
| LMDDx      = [[DLBCL]], intermediate DLBCL/BL
| Stains    =  
| Stains    =
| IHC        = CD20+, CD10+, BCL6+, EBER+, Ki-67 (100%)
| IHC        = CD20+, CD10+, BCL6+, EBER+, Ki-67 (100%)
| EM        =
| EM        =
Line 14: Line 14:
| Gross      =
| Gross      =
| Grossing  =
| Grossing  =
| Site      = [[Lymph node]]
| Site      = [[lymph node]]
| Assdx      = +/-[[EBV]] infection, +/-[[HIV]] infection
| Syndromes  =
| Clinicalhx = very rapid growth
| Signs      =
| Signs      =
| Symptoms  =
| Symptoms  =
| Prevalence = Uncommon
| Prevalence = uncommon
| Assdx      = +/-[[EBV]] infection, +/-[[HIV]] infection
| Syndromes  =
| Bloodwork  =
| Bloodwork  =
| Rads      =
| Rads      =
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| ClinDDx    =
| ClinDDx    =
}}
}}
'''Burkitt lymphoma''', abbreviated '''BL''', is an uncommon [[lymphoma]] with medium-sized cells.
'''Burkitt lymphoma''', abbreviated '''BL''', is an uncommon [[lymphoma]] with medium-sized cells.



Revision as of 20:41, 5 July 2013

Burkitt lymphoma
Diagnosis in short

Starry-sky pattern. H&E stain.

LM medium-sized lymphoid cells of uniform size with abundant cytoplasm, interspersed macrophages
Subtypes endemic, non-endemic, immunodeficiency associated
LM DDx DLBCL, intermediate DLBCL/BL
IHC CD20+, CD10+, BCL6+, EBER+, Ki-67 (100%)
Molecular t(8;14) (q24;q32) + others
Site lymph node

Associated Dx +/-EBV infection, +/-HIV infection
Clinical history very rapid growth
Prevalence uncommon

Burkitt lymphoma, abbreviated BL, is an uncommon lymphoma with medium-sized cells.

General

  • Extremely high proliferative rate & rate of apoptosis.
  • Rare in adults -- 1-2% of adult lymphomas.[1]
  • Relatively common in children -- 30-50% of childhood lymphomas.[1]

Pathophysiology

  • Origin cell: germinal centre B cells (favoured) vs. memory B cells.[2]

Subtypes

  • Three subtypes recognized:[2]
  1. Endemic:
  2. Non-endemic:
    • Typical of the BL seen in the western world; EBV negative.
  3. Immunodeficiency associated:
    • Associated with HIV infection.

Microscopic

Features:

  • "Starry-sky pattern":
    • The stars in the pattern are: tingible-body macrophages.
      • Tingible-body macrophages = macrophages containing apoptotic tumour cells.
    • The tumour cells are the sky.
  • Tumour cells:[2]
    • Medium-sized (~1.5-2x the size of a RBC) with uniform size ("monotonous") -- key feature.
    • Round nucleus.
    • Small nucleoli.
    • Relatively abundant cytoplasm.
    • Brisk mitotic rate.

DDx:

  • DLBCL.
  • B cell lymphoma unclassifiable with features intermediate between DLBCL and Burkitt lymphoma.[3]
    • May be referred to as intermediate DLBCL/BL.[4]

Images

www:

Cytology

Features:

  • Medium-sized lymphoid cells with small lipid vacuoles (~1-2 micrometers).[5]

Image:

IHC

Features:

  • CD20 +ve.
  • CD10 +ve.
  • BCL6 +ve.
  • EBER +ve.
  • Ki-67 ~100%.

Others:

  • BCL2 -ve.

Molecular pathology

Note:

  • MYC rearrangement is typically assessed with an ISH break apart probe.

See also

References

  1. Jump up to: 1.0 1.1 1.2 Online 'Mendelian Inheritance in Man' (OMIM) 113970
  2. Jump up to: 2.0 2.1 2.2 2.3 2.4 2.5 Bellan C, Lazzi S, De Falco G, Nyongo A, Giordano A, Leoncini L (March 2003). "Burkitt's lymphoma: new insights into molecular pathogenesis". J. Clin. Pathol. 56 (3): 188–92. PMC 1769902. PMID 12610094. http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=12610094.
  3. Carbone, A.; Gloghini, A.; Aiello, A.; Testi, A.; Cabras, A. (May 2010). "B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology.". Hum Pathol 41 (5): 621-31. doi:10.1016/j.humpath.2009.10.027. PMID 20398809.
  4. Ahn, JY.; Seo, YH.; Park, PW.; Kim, KH.; Park, MJ.; Jeong, JH.; Park, SH.; Song, YH. (Mar 2012). "A case of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma in a Korean child.". Ann Lab Med 32 (2): 162-6. doi:10.3343/alm.2012.32.2.162. PMID 22389885.
  5. Troxell, ML.; Bangs, CD.; Cherry, AM.; Natkunam, Y.; Kong, CS. (Oct 2005). "Cytologic diagnosis of Burkitt lymphoma.". Cancer 105 (5): 310-8. doi:10.1002/cncr.21307. PMID 15986398.