HAEM5:Plasmablastic lymphoma: Difference between revisions

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Fabiola Quintero-Rivera, MD (University of California, Irvine)

==WHO Classification of Disease==

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''MYC'' (8q24) up-regulation occurs via translocations, frequently between ''MYC'' and immunoglobulin (''IG)'' heavy chain [t(8;14)] and immunoglobulin light chain genes [t(2;8) or t(8;22)], which are also seen in Burkitt lymphoma. These translocations are more common in EBV-positive tumors (74%), and have been associated with poorer prognosis<ref>{{Cite journal|last=Bogusz|first=Agata M.|last2=Seegmiller|first2=Adam C.|last3=Garcia|first3=Rolando|last4=Shang|first4=Ping|last5=Ashfaq|first5=Raheela|last6=Chen|first6=Weina|date=2009|title=Plasmablastic lymphomas with MYC/IgH rearrangement: report of three cases and review of the literature|url=https://www.ncbi.nlm.nih.gov/pubmed/19762538|journal=American Journal of Clinical Pathology|volume=132|issue=4|pages=597–605|doi=10.1309/AJCPFUR1BK0UODTS|issn=1943-7722|pmid=19762538}}</ref><ref name=":5">{{Cite journal|last=Valera|first=Alexandra|last2=Balagué|first2=Olga|last3=Colomo|first3=Luis|last4=Martínez|first4=Antonio|last5=Delabie|first5=Jan|last6=Taddesse-Heath|first6=Lekidelu|last7=Jaffe|first7=Elaine S.|last8=Campo|first8=Elías|date=2010|title=IG/MYC rearrangements are the main cytogenetic alteration in plasmablastic lymphomas|url=https://www.ncbi.nlm.nih.gov/pubmed/20962620|journal=The American Journal of Surgical Pathology|volume=34|issue=11|pages=1686–1694|doi=10.1097/PAS.0b013e3181f3e29f|issn=1532-0979|pmc=2982261|pmid=20962620}}</ref>.

* Chromosomal Rearrangements (Gene Fusions)

* Individual Region Genomic Gain/Loss/LOH

* Gene Mutations (SNV/INDEL)}}</blockquote>

*There is currently no standard therapy for PBL. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) have been generally considered inadequate, and the National Comprehensive Cancer Network (NCCN) favors Hyper-CVAD-MA (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone, and high-dose methotrexate and cytarabine), CODOX-M/IVAC (cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, and high-dose cytarabine), COMB (cyclophosphamide, Oncovin, methyl-CCNU, and bleomycin), and infusional EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin)<ref name=":5" /><ref>{{Cite journal|last=Zelenetz|first=Andrew D.|last2=Abramson|first2=Jeremy S.|last3=Advani|first3=Ranjana H.|last4=Andreadis|first4=C. Babis|last5=Bartlett|first5=Nancy|last6=Bellam|first6=Naresh|last7=Byrd|first7=John C.|last8=Czuczman|first8=Myron S.|last9=Fayad|first9=Luis E.|date=2011|title=Non-Hodgkin's lymphomas|url=https://www.ncbi.nlm.nih.gov/pubmed/21550968|journal=Journal of the National Comprehensive Cancer Network: JNCCN|volume=9|issue=5|pages=484–560|doi=10.6004/jnccn.2011.0046|issn=1540-1413|pmid=21550968}}</ref><ref>{{Cite journal|last=Koizumi|first=Yusuke|last2=Uehira|first2=Tomoko|last3=Ota|first3=Yasunori|last4=Ogawa|first4=Yoshihiko|last5=Yajima|first5=Keishiro|last6=Tanuma|first6=Junko|last7=Yotsumoto|first7=Mihoko|last8=Hagiwara|first8=Shotaro|last9=Ikegaya|first9=Satoshi|date=2016|title=Clinical and pathological aspects of human immunodeficiency virus-associated plasmablastic lymphoma: analysis of 24 cases|url=https://www.ncbi.nlm.nih.gov/pubmed/27604616|journal=International Journal of Hematology|volume=104|issue=6|pages=669–681|doi=10.1007/s12185-016-2082-3|issn=1865-3774|pmid=27604616}}</ref>.

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One study of 12 PBL cases showed recurrent gains of 1q31.1q44, 5p15.33p13.1, 7q11.2q11.23, 8q24.13q24.3, 11p and 11q terminal regions, 15q15q26.3, 19p13.3p13.12 and chromosomes 3, 7, 11, and 15, as well as losses of 1p36.33p35.1, 6q25.1q27, 8p23.3p22.14 and 18q21.32q23. Additionally, 54% of the cases had either deletion or copy neutral loss of heterozygosity (CN-LOH) involving the tumor suppressor gene ''CDKN2C'' at 1p32.3. Furthermore, recurrent copy number losses involving the immunoglobulin genes ''IGH'' and ''IGKV'' were documented<ref>{{Cite journal|last=Ji|first=Jianling|last2=Quintero-Rivera|first2=Fabiola|last3=Xian|first3=Rena|last4=Crane|first4=Genevieve|last5=Baden|first5=Kevin|last6=Moschiano|first6=Elizabeth|last7=Karunasiri|first7=Deepthi K.|last8=Duffield|first8=Amy Susan|last9=Rao|first9=Nagesh|date=2016-06|title=Genomic Profiling of Plasmablastic Lymphoma Reveals Recurrent Copy Number Alterations and MYC Rearrangement as Common Genetic Abnormalities|url=https://doi.org/10.1016/j.cancergen.2016.04.021|journal=Cancer Genetics|volume=209|issue=6|pages=290|doi=10.1016/j.cancergen.2016.04.021|issn=2210-7762}}</ref>.

!Chromosomal Pattern

!Molecular Pathogenesis

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|<span class="blue-text">EXAMPLE:</span>''EGFR''

|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.

*''IGHV'' may be unmutated or demonstrate somatic hypermutation<ref>{{Cite journal|last=Gaidano|first=Gianluca|last2=Cerri|first2=Michaela|last3=Capello|first3=Daniela|last4=Berra|first4=Eva|last5=Deambrogi|first5=Clara|last6=Rossi|first6=Davide|last7=Larocca|first7=Luigi Maria|last8=Campo|first8=Elias|last9=Gloghini|first9=Annunziata|date=2002|title=Molecular histogenesis of plasmablastic lymphoma of the oral cavity|url=https://www.ncbi.nlm.nih.gov/pubmed/12437635|journal=British Journal of Haematology|volume=119|issue=3|pages=622–628|doi=10.1046/j.1365-2141.2002.03872.x|issn=0007-1048|pmid=12437635}}</ref>.

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*''MYC'' expression is suppressed by ''PRDM1 (''BLIMP1) in terminally differentiated B cells; ''BLIMP1'' encodes a transcriptional factor responsible for plasma cell differentiation<ref name=":5" />.

(use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span> <references />

==Notes==

          

<nowiki>*</nowiki>''Citation of this Page'': “Plasmablastic lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Plasmablastic_lymphoma</nowiki>.