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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 = | ||
| Molecular = t(8;14) (q24;q32) + others | | Molecular = t(8;14) (q24;q32) + others | ||
| Line 14: | Line 14: | ||
| Gross = | | Gross = | ||
| Grossing = | | Grossing = | ||
| Site = [[ | | Site = [[lymph node]] | ||
| Assdx = +/-[[EBV]] infection, +/-[[HIV]] infection | |||
| Syndromes = | |||
| Clinicalhx = very rapid growth | |||
| Signs = | | Signs = | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = | | Prevalence = uncommon | ||
| 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. | ||
==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> | *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> | *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=== | ===Pathophysiology=== | ||
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#*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 | |||
==Microscopic== | ==Microscopic== | ||
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**Small nucleoli. | **Small nucleoli. | ||
**Relatively abundant cytoplasm. | **Relatively abundant cytoplasm. | ||
**Brisk mitotic rate. | **Brisk mitotic rate (near 100%). | ||
DDx: | DDx: | ||
| Line 79: | Line 83: | ||
==IHC== | ==IHC== | ||
Features: | Features: | ||
*CD20 +ve. | *[[CD20]] +ve. | ||
*CD10 +ve. | *CD10 +ve. | ||
*BCL6 +ve. | *BCL6 +ve. | ||
*EBER +ve. | *[[EBER]] +ve. | ||
*Ki-67 ~100%. | *Ki-67 ~100%. | ||
Others: | Others: | ||
*BCL2 -ve | *BCL2 -ve (may show weak BCL2 | ||
==Molecular pathology== | ==Molecular pathology== | ||
*t(8;14) (q24;q32) | *Isolated CMYC-IGH [[translocation]] - t(8;14) (q24;q32).<ref name=pmid12610094/> | ||
*Several other variants -- involve MYC rearrangement.<ref name=pmid12610094/><ref name=omim113970>{{OMIM|113970}}</ref> | *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 | |||
Note: | Note: | ||