Difference between revisions of "Burkitt lymphoma"

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'''Burkitt lymphoma''', abbreviated '''BL''', is an uncommon medium cell [[lymphoma]].
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Burkitt_lymphoma,_H%26E.jpg
| Width      =
| Caption    = Starry-sky pattern. [[H&E stain]].
| Micro      = medium-sized lymphoid cells of uniform size with abundant cytoplasm, interspersed macrophages
| Subtypes  = endemic, non-endemic, immunodeficiency associated
| LMDDx      = [[DLBCL]], intermediate DLBCL/BL
| Stains    =
| IHC        = [[CD20]]+, CD10+, BCL6+, [[EBER]]+, Ki-67 (100%)
| EM        =
| Molecular  = t(8;14) (q24;q32) + others
| IF        =
| Gross      =
| Grossing  =
| Site      = [[lymph node]]
| Assdx      = +/-[[EBV]] infection, +/-[[HIV]] infection
| Syndromes  =
| Clinicalhx = very rapid growth
| Signs      =
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
}}
'''Burkitt lymphoma''', abbreviated '''BL''', is an uncommon [[lymphoma]] with medium-sized cells.


==General==
==General==
*Extremely high proliferative rate & rate of apoptosis.
*Extremely high proliferative rate & rate of [[apoptosis]]
*Rare in adults -- 1-2% of adult lymphomas.<ref name=omim113970>{{OMIM|113970}}</ref>
*Relatively common in children -- 30-50% of childhood lymphomas.<ref name=omim113970>{{OMIM|113970}}</ref>
*Although aggressive untreated, the high mitotic activity makes it relatively chemosensitive and complete remissions of 75-90% are described
 
===Pathophysiology===
*Origin cell: germinal centre B cells (favoured) vs. memory B cells.<ref name=pmid12610094/>


==Subtypes==
==Subtypes==
Line 8: Line 43:
#Endemic:
#Endemic:
#*Found in Africa.
#*Found in Africa.
#*EBV (Epstein-Barr virus) associated.<ref name=pmid12610094/>
#*EBV ([[Epstein-Barr virus]]) associated.<ref name=pmid12610094/>
#Non-endemic:
#Non-endemic/sporadic:
#*Typical of the BL seen in the western world; EBV negative.
#*Typical of the BL seen in the western world; EBV negative.
#Immunodeficiency associated:
#Immunodeficiency associated:
#*Associated with HIV infection.
#*Associated with [[HIV]] infection.
 
#*Post-transplantation immunosuppression
==Pathophysiology==
*Origin cell: germinal centre B cells (favoured) vs. memory B cells.<ref name=pmid12610094/>
*Common translocation t(8;14).<ref name=pmid12610094/>
 
==Cytologic definition==
*t(8;14) (q24;q32) translocation + a few variants or c-myc rearrangement.<ref name=pmid12610094/>


==Microscopic==
==Microscopic==
Features:
Features:
*"Starry-sky pattern".
*"Starry-sky pattern":
**The ''stars'' in the pattern are: tingible-bodies laden macrophages.  
**The ''stars'' in the pattern are: tingible-body macrophages.  
***''[[Tingible-bodies macrophages]]'' = macrophages containing dead body of apoptotic tumor cells.  
***''Tingible-body macrophages'' = macrophages containing apoptotic tumour cells.  
**The tumour cells are the sky.
**The tumour cells are the sky.
*Tumour cells:<ref name=pmid12610094/>
*Tumour cells:<ref name=pmid12610094/>
**Medium size cells (~1.5-2x the size of a RBC) with uniform size ("monotonous") -- '''key feature'''.
**Medium-sized (~1.5-2x the size of a RBC) with uniform size ("monotonous") -- '''key feature'''.
**Round nuclei.
**Round nucleus.
**Small nucleolus.
**Small nucleoli.
**Relatively abundant cytoplasm.
**Relatively abundant cytoplasm.
**Brisk mitotic rate.
**Brisk mitotic rate (near 100%).
 
DDx:
*[[DLBCL]].
*B cell lymphoma unclassifiable with features intermediate between DLBCL and Burkitt lymphoma.<ref name=pmid20398809>{{Cite journal  | last1 = Carbone | first1 = A. | last2 = Gloghini | first2 = A. | last3 = Aiello | first3 = A. | last4 = Testi | first4 = A. | last5 = Cabras | first5 = A. | title = B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology. | journal = Hum Pathol | volume = 41 | issue = 5 | pages = 621-31 | month = May | year = 2010 | doi = 10.1016/j.humpath.2009.10.027 | PMID = 20398809 }}</ref>
**May be referred to as ''intermediate DLBCL/BL''.<ref name=pmid22389885>{{Cite journal  | last1 = Ahn | first1 = JY. | last2 = Seo | first2 = YH. | last3 = Park | first3 = PW. | last4 = Kim | first4 = KH. | last5 = Park | first5 = MJ. | last6 = Jeong | first6 = JH. | last7 = Park | first7 = SH. | last8 = Song | first8 = YH. | title = A case of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma in a Korean child. | journal = Ann Lab Med | volume = 32 | issue = 2 | pages = 162-6 | month = Mar | year = 2012 | doi = 10.3343/alm.2012.32.2.162 | PMID = 22389885 }}</ref>
===Images===
<gallery>
Image:Burkitt_lymphoma,_H%26E.jpg | Starry-sky pattern. (WC/Uthman)
</gallery>
www:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1769902/figure/f1/ Burkitt lymphoma (nih.gov)].
 
==Cytology==
Features:
*Medium-sized lymphoid cells with small lipid vacuoles (~1-2 micrometers).<ref name=pmid15986398>{{Cite journal  | last1 = Troxell | first1 = ML. | last2 = Bangs | first2 = CD. | last3 = Cherry | first3 = AM. | last4 = Natkunam | first4 = Y. | last5 = Kong | first5 = CS. | title = Cytologic diagnosis of Burkitt lymphoma. | journal = Cancer | volume = 105 | issue = 5 | pages = 310-8 | month = Oct | year = 2005 | doi = 10.1002/cncr.21307 | PMID = 15986398 }}</ref>
 
Image:
*[http://commons.wikimedia.org/wiki/File:Burkitt_lymphoma,_touch_prep,_Wright_stain.jpg Burkitt lymphoma (WC/Uthman)].
 
==IHC==
Features:
*[[CD20]] +ve.
*CD10 +ve.
*BCL6 +ve.
*[[EBER]] +ve.
*Ki-67 ~100%.
 
Others:
*BCL2 -ve (may show weak BCL2
 
==Molecular pathology==
*Isolated CMYC-IGH [[translocation]] - t(8;14) (q24;q32).<ref name=pmid12610094/>
*Several other variants -- involve MYC rearrangement with other partners.<ref name=pmid12610094/><ref name=omim113970>{{OMIM|113970}}</ref>
*Should not have CCND1, BCL6 or BCL2 translocations


Image: [http://en.wikipedia.org/wiki/File:Burkitt_lymphoma,_H%26E.jpg Starry-sky pattern - Ed Uthman (WC)].
Note:
*MYC rearrangement is typically assessed with an [[ISH]] break apart probe.


==See also==
==See also==
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[[Category:Haematopathology]]
[[Category:Haematopathology]]
[[Category:Diagnosis]]

Latest revision as of 19:58, 26 December 2019

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]
  • Although aggressive untreated, the high mitotic activity makes it relatively chemosensitive and complete remissions of 75-90% are described

Pathophysiology

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

Subtypes

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

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 (near 100%).

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

  • Starry-sky pattern. (WC/Uthman)

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 (may show weak BCL2

Molecular pathology

  • Isolated CMYC-IGH translocation - t(8;14) (q24;q32).[2]
  • Several other variants -- involve MYC rearrangement with other partners.[2][1]
  • Should not have CCND1, BCL6 or BCL2 translocations

Note:

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

See also

References

  1. ↑ 1.0 1.1 1.2 Online 'Mendelian Inheritance in Man' (OMIM) 113970
  2. ↑ 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.
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