HAEM5:Systemic EBV-positive T-cell lymphoma of childhood: Difference between revisions

[unchecked revision][unchecked revision]
No edit summary
Removed old template contents
Line 1: Line 1:
{{DISPLAYTITLE:Systemic EBV-positive T-cell lymphoma of childhood}}
{{DISPLAYTITLE:Systemic EBV-positive T-cell lymphoma of childhood}}
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]


{{Under Construction}}
{{Under Construction}}


<blockquote class='blockedit'>{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Systemic EBV-Positive T-cell Lymphoma of Childhood]].
<blockquote class="blockedit">{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Systemic EBV-Positive T-cell Lymphoma of Childhood]].
}}</blockquote>
}}</blockquote>


Line 37: Line 38:
|}
|}


==Definition / Description of Disease==
*A life-threatening clonal disease resulting from primary Epstein-Barr virus (EBV) infected T-cells or in the setting of systemic chronic active EBV infection (CAEBV)
*T-lymphocytes infected with EBV infiltrate the liver, spleen, lungs, skin and marrow, resulting in multiorgan failure, sepsis and death
*Rapidly progressive
*Most common in children and young adults after a primary EBV infection; can occur in adult patients
<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":0">{{Cite journal|last=Kimura|first=H.|last2=Hoshino|first2=Y.|last3=Kanegane|first3=H.|last4=Tsuge|first4=I.|last5=Okamura|first5=T.|last6=Kawa|first6=K.|last7=Morishima|first7=T.|date=2001-07-15|title=Clinical and virologic characteristics of chronic active Epstein-Barr virus infection|url=https://pubmed.ncbi.nlm.nih.gov/11435294|journal=Blood|volume=98|issue=2|pages=280–286|doi=10.1182/blood.v98.2.280|issn=0006-4971|pmid=11435294}}</ref><ref name=":1">{{Cite journal|last=Quintanilla-Martinez|first=L.|last2=Kumar|first2=S.|last3=Fend|first3=F.|last4=Reyes|first4=E.|last5=Teruya-Feldstein|first5=J.|last6=Kingma|first6=D. W.|last7=Sorbara|first7=L.|last8=Raffeld|first8=M.|last9=Straus|first9=S. E.|date=2000-07-15|title=Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome|url=https://pubmed.ncbi.nlm.nih.gov/10887104|journal=Blood|volume=96|issue=2|pages=443–451|issn=0006-4971|pmid=10887104}}</ref><ref name=":2">{{Cite journal|last=Kikuta|first=H.|last2=Sakiyama|first2=Y.|last3=Matsumoto|first3=S.|last4=Oh-Ishi|first4=T.|last5=Nakano|first5=T.|last6=Nagashima|first6=T.|last7=Oka|first7=T.|last8=Hironaka|first8=T.|last9=Hirai|first9=K.|date=1993-12-01|title=Fatal Epstein-Barr virus-associated hemophagocytic syndrome|url=https://pubmed.ncbi.nlm.nih.gov/8241498|journal=Blood|volume=82|issue=11|pages=3259–3264|issn=0006-4971|pmid=8241498}}</ref><ref name=":3">{{Cite journal|last=Su|first=I. J.|last2=Chen|first2=R. L.|last3=Lin|first3=D. T.|last4=Lin|first4=K. S.|last5=Chen|first5=C. C.|date=1994-06|title=Epstein-Barr virus (EBV) infects T lymphocytes in childhood EBV-associated hemophagocytic syndrome in Taiwan|url=https://pubmed.ncbi.nlm.nih.gov/8203462|journal=The American Journal of Pathology|volume=144|issue=6|pages=1219–1225|issn=0002-9440|pmc=1887465|pmid=8203462}}</ref><ref name=":4">{{Cite journal|last=Suzuki|first=Keiko|last2=Ohshima|first2=Koichi|last3=Karube|first3=Kennosuke|last4=Suzumiya|first4=Junji|last5=Ohga|first5=Shouichi|last6=Ishihara|first6=Shigehiko|last7=Tamura|first7=Kazuo|last8=Kikuchi|first8=Masahiro|date=2004-05|title=Clinicopathological states of Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorders (severe chronic active EBV infection) of children and young adults|url=https://pubmed.ncbi.nlm.nih.gov/15067338|journal=International Journal of Oncology|volume=24|issue=5|pages=1165–1174|issn=1019-6439|pmid=15067338}}</ref><ref name=":5">{{Cite journal|last=Hue|first=Susan Swee-Shan|last2=Oon|first2=Ming Liang|last3=Wang|first3=Shi|last4=Tan|first4=Soo-Yong|last5=Ng|first5=Siok-Bian|date=2020-01|title=Epstein-Barr virus-associated T- and NK-cell lymphoproliferative diseases: an update and diagnostic approach|url=https://pubmed.ncbi.nlm.nih.gov/31767131|journal=Pathology|volume=52|issue=1|pages=111–127|doi=10.1016/j.pathol.2019.09.011|issn=1465-3931|pmid=31767131}}</ref><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==Synonyms / Terminology==
*Fulminant EBV-positive T-cell lymphoproliferative disorder of childhood
*Sporadic fatal infectious mononucleosis
*Fulminant hemophagocytic syndrome in children in Taiwan
*Fatal EBV-associated hemophagocytic syndrome
*Severe Chronic Active EBV Infection (CAEBV; legacy term)
<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":5" /><ref>{{Cite journal|last=Ohshima|first=Koichi|last2=Kimura|first2=Hiroshi|last3=Yoshino|first3=Tadashi|last4=Kim|first4=Chul Woo|last5=Ko|first5=Young H.|last6=Lee|first6=Seung-Suk|last7=Peh|first7=Suat-Cheng|last8=Chan|first8=John K. C.|last9=CAEBV Study Group|date=2008-04|title=Proposed categorization of pathological states of EBV-associated T/natural killer-cell lymphoproliferative disorder (LPD) in children and young adults: overlap with chronic active EBV infection and infantile fulminant EBV T-LPD|url=https://pubmed.ncbi.nlm.nih.gov/18324913|journal=Pathology International|volume=58|issue=4|pages=209–217|doi=10.1111/j.1440-1827.2008.02213.x|issn=1440-1827|pmid=18324913}}</ref><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==Epidemiology / Prevalence==
*Most prevalent in Asia (Japan and Taiwan)
*Has been reported in Mexico, Central and South America
*Rare in Western countries
*Children and young adults
*No sex predilection
<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":3" /><ref name=":4" /><ref>{{Cite journal|last=Kimura|first=Hiroshi|last2=Morishima|first2=Tsuneo|last3=Kanegane|first3=Hirokazu|last4=Ohga|first4=Shouichi|last5=Hoshino|first5=Yo|last6=Maeda|first6=Akihiko|last7=Imai|first7=Shosuke|last8=Okano|first8=Motohiko|last9=Morio|first9=Tomohiro|date=2003-02-15|title=Prognostic factors for chronic active Epstein-Barr virus infection|url=https://pubmed.ncbi.nlm.nih.gov/12599068|journal=The Journal of Infectious Diseases|volume=187|issue=4|pages=527–533|doi=10.1086/367988|issn=0022-1899|pmid=12599068}}</ref><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==Clinical Features==
Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
{| class="wikitable"
|'''Signs and Symptoms'''
|<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)
<span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)
<span class="blue-text">EXAMPLE:</span> Fatigue
<span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
|-
|'''Laboratory Findings'''
|<span class="blue-text">EXAMPLE:</span> Cytopenias
<span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
|}
<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}</blockquote>
Signs & Symptoms <ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":3" /><ref name=":4" /><ref name=":6">{{Cite journal|last=Jones|first=J. F.|last2=Shurin|first2=S.|last3=Abramowsky|first3=C.|last4=Tubbs|first4=R. R.|last5=Sciotto|first5=C. G.|last6=Wahl|first6=R.|last7=Sands|first7=J.|last8=Gottman|first8=D.|last9=Katz|first9=B. Z.|date=1988-03-24|title=T-cell lymphomas containing Epstein-Barr viral DNA in patients with chronic Epstein-Barr virus infections|url=https://pubmed.ncbi.nlm.nih.gov/2831453|journal=The New England Journal of Medicine|volume=318|issue=12|pages=733–741|doi=10.1056/NEJM198803243181203|issn=0028-4793|pmid=2831453}}</ref><ref name=":7">{{Cite journal|last=Kanegane|first=H.|last2=Bhatia|first2=K.|last3=Gutierrez|first3=M.|last4=Kaneda|first4=H.|last5=Wada|first5=T.|last6=Yachie|first6=A.|last7=Seki|first7=H.|last8=Arai|first8=T.|last9=Kagimoto|first9=S.|date=1998-03-15|title=A syndrome of peripheral blood T-cell infection with Epstein-Barr virus (EBV) followed by EBV-positive T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/9490694|journal=Blood|volume=91|issue=6|pages=2085–2091|issn=0006-4971|pmid=9490694}}</ref>
*Acute onset fever that is unresponsive to antibiotics
*General malaise
*Splenic and liver enlargement
*Liver failure/jaundice
*Lymphadenopathy (uncommon)
Laboratory Findings <ref name=":1" /><ref name=":4" />
*Pancytopenia
*Abnormal liver function tests
*Abnormal EBV serology with low or absent anti-VCA IgM antibodies
*Hemophagocytic syndrome (coagulopathy, multiorgan failure and sepsis)
*CAEBV infection (in some cases)
<blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==Sites of Involvement==
Systemic disease with most commonly involved sites:
*Liver
*Spleen
*Lymph nodes
*Bone marrow
*Skin
*Lung
<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":5" /><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==Morphologic Features==
*Small T-cells
*Medium to large lymphoid cells with irregular nuclei and frequent mitoses (less common)
*Sinusoidal infiltration of liver and spleen with hemophagocytosis
*Spleen: depleted white pulp
*Liver: prominent portal and sinusoidal infiltration, cholestasis, steatosis and necrosis
*Lymph nodes: preserved architecture, sinus histiocytosis and erythrophagocytosis
*Bone marrow: histiocytic hyperplasia and erythrophagocytosis
<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":4" /><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==Immunophenotype==
<br />
{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive||CD2, CD3, TIA1, CD8 (''de novo'' EBV infection), CD4 (severe CAEBV)
|-
|Negative||CD56
|}
<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":3" /><ref name=":5" /><ref name=":6" /><ref name=":7" /><ref name=":8">{{Cite journal|last=Kasahara|first=Y.|last2=Yachie|first2=A.|last3=Takei|first3=K.|last4=Kanegane|first4=C.|last5=Okada|first5=K.|last6=Ohta|first6=K.|last7=Seki|first7=H.|last8=Igarashi|first8=N.|last9=Maruhashi|first9=K.|date=2001-09-15|title=Differential cellular targets of Epstein-Barr virus (EBV) infection between acute EBV-associated hemophagocytic lymphohistiocytosis and chronic active EBV infection|url=https://pubmed.ncbi.nlm.nih.gov/11535525|journal=Blood|volume=98|issue=6|pages=1882–1888|doi=10.1182/blood.v98.6.1882|issn=0006-4971|pmid=11535525}}</ref><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==WHO Essential and Desirable Genetic Diagnostic Criteria==
==WHO Essential and Desirable Genetic Diagnostic Criteria==
<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span>
<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span>
Line 259: Line 126:
|}
|}


<blockquote class='blockedit'>{{Box-round|title=v4:Chromosomal Rearrangements (Gene Fusions)|The content below was from the old template. Please incorporate above.}}</blockquote>
<blockquote class="blockedit">{{Box-round|title=v4:Chromosomal Rearrangements (Gene Fusions)|The content below was from the old template. Please incorporate above.}}</blockquote>


*No reported gene fusions
*No reported gene fusions
Line 269: Line 136:




<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:
<blockquote class="blockedit">{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:
* Chromosomal Rearrangements (Gene Fusions)
* Chromosomal Rearrangements (Gene Fusions)
* Individual Region Genomic Gain/Loss/LOH
* Individual Region Genomic Gain/Loss/LOH
Line 337: Line 204:
|}
|}


<blockquote class='blockedit'>{{Box-round|title=v4:Genomic Gain/Loss/LOH|The content below was from the old template. Please incorporate above.}}</blockquote>
<blockquote class="blockedit">{{Box-round|title=v4:Genomic Gain/Loss/LOH|The content below was from the old template. Please incorporate above.}}</blockquote>


*N/A
*N/A
Line 383: Line 250:
|}
|}


<blockquote class='blockedit'>{{Box-round|title=v4:Characteristic Chromosomal Aberrations / Patterns|The content below was from the old template. Please incorporate above.}}</blockquote>
<blockquote class="blockedit">{{Box-round|title=v4:Characteristic Chromosomal Aberrations / Patterns|The content below was from the old template. Please incorporate above.}}</blockquote>




Line 390: Line 257:




<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":4" /><ref name=":5" /><ref name=":8" /><ref>{{Cite journal|last=Au|first=W.-Y.|last2=Ma|first2=S.-Y.|last3=Chim|first3=C.-S.|last4=Choy|first4=C.|last5=Loong|first5=F.|last6=Lie|first6=A. K. W.|last7=Lam|first7=C. C. K.|last8=Leung|first8=A. Y. H.|last9=Tse|first9=E.|date=2005-02|title=Clinicopathologic features and treatment outcome of mature T-cell and natural killer-cell lymphomas diagnosed according to the World Health Organization classification scheme: a single center experience of 10 years|url=https://pubmed.ncbi.nlm.nih.gov/15668271|journal=Annals of Oncology: Official Journal of the European Society for Medical Oncology|volume=16|issue=2|pages=206–214|doi=10.1093/annonc/mdi037|issn=0923-7534|pmid=15668271}}</ref><ref name=":9">{{Cite journal|last=Smith|first=Megan C.|last2=Cohen|first2=Daniel N.|last3=Greig|first3=Bruce|last4=Yenamandra|first4=Ashwini|last5=Vnencak-Jones|first5=Cindy|last6=Thompson|first6=Mary Ann|last7=Kim|first7=Annette S.|date=2014|title=The ambiguous boundary between EBV-related hemophagocytic lymphohistiocytosis and systemic EBV-driven T cell lymphoproliferative disorder|url=https://pubmed.ncbi.nlm.nih.gov/25337215|journal=International Journal of Clinical and Experimental Pathology|volume=7|issue=9|pages=5738–5749|issn=1936-2625|pmc=4203186|pmid=25337215}}</ref><ref>{{Cite journal|last=Chen|first=J. S.|last2=Tzeng|first2=C. C.|last3=Tsao|first3=C. J.|last4=Su|first4=W. C.|last5=Chen|first5=T. Y.|last6=Jung|first6=Y. C.|last7=Su|first7=I. J.|date=1997-09|title=Clonal karyotype abnormalities in EBV-associated hemophagocytic syndrome|url=https://pubmed.ncbi.nlm.nih.gov/9407723|journal=Haematologica|volume=82|issue=5|pages=572–576|issn=0390-6078|pmid=9407723}}</ref><blockquote class="blockedit">
<blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":0">{{Cite journal|last=Kimura|first=H.|last2=Hoshino|first2=Y.|last3=Kanegane|first3=H.|last4=Tsuge|first4=I.|last5=Okamura|first5=T.|last6=Kawa|first6=K.|last7=Morishima|first7=T.|date=2001-07-15|title=Clinical and virologic characteristics of chronic active Epstein-Barr virus infection|url=https://pubmed.ncbi.nlm.nih.gov/11435294|journal=Blood|volume=98|issue=2|pages=280–286|doi=10.1182/blood.v98.2.280|issn=0006-4971|pmid=11435294}}</ref><ref name=":1">{{Cite journal|last=Quintanilla-Martinez|first=L.|last2=Kumar|first2=S.|last3=Fend|first3=F.|last4=Reyes|first4=E.|last5=Teruya-Feldstein|first5=J.|last6=Kingma|first6=D. W.|last7=Sorbara|first7=L.|last8=Raffeld|first8=M.|last9=Straus|first9=S. E.|date=2000-07-15|title=Fulminant EBV(+) T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome|url=https://pubmed.ncbi.nlm.nih.gov/10887104|journal=Blood|volume=96|issue=2|pages=443–451|issn=0006-4971|pmid=10887104}}</ref><ref name=":2">{{Cite journal|last=Kikuta|first=H.|last2=Sakiyama|first2=Y.|last3=Matsumoto|first3=S.|last4=Oh-Ishi|first4=T.|last5=Nakano|first5=T.|last6=Nagashima|first6=T.|last7=Oka|first7=T.|last8=Hironaka|first8=T.|last9=Hirai|first9=K.|date=1993-12-01|title=Fatal Epstein-Barr virus-associated hemophagocytic syndrome|url=https://pubmed.ncbi.nlm.nih.gov/8241498|journal=Blood|volume=82|issue=11|pages=3259–3264|issn=0006-4971|pmid=8241498}}</ref><ref name=":4">{{Cite journal|last=Suzuki|first=Keiko|last2=Ohshima|first2=Koichi|last3=Karube|first3=Kennosuke|last4=Suzumiya|first4=Junji|last5=Ohga|first5=Shouichi|last6=Ishihara|first6=Shigehiko|last7=Tamura|first7=Kazuo|last8=Kikuchi|first8=Masahiro|date=2004-05|title=Clinicopathological states of Epstein-Barr virus-associated T/NK-cell lymphoproliferative disorders (severe chronic active EBV infection) of children and young adults|url=https://pubmed.ncbi.nlm.nih.gov/15067338|journal=International Journal of Oncology|volume=24|issue=5|pages=1165–1174|issn=1019-6439|pmid=15067338}}</ref><ref name=":5">{{Cite journal|last=Hue|first=Susan Swee-Shan|last2=Oon|first2=Ming Liang|last3=Wang|first3=Shi|last4=Tan|first4=Soo-Yong|last5=Ng|first5=Siok-Bian|date=2020-01|title=Epstein-Barr virus-associated T- and NK-cell lymphoproliferative diseases: an update and diagnostic approach|url=https://pubmed.ncbi.nlm.nih.gov/31767131|journal=Pathology|volume=52|issue=1|pages=111–127|doi=10.1016/j.pathol.2019.09.011|issn=1465-3931|pmid=31767131}}</ref><ref name=":8">{{Cite journal|last=Kasahara|first=Y.|last2=Yachie|first2=A.|last3=Takei|first3=K.|last4=Kanegane|first4=C.|last5=Okada|first5=K.|last6=Ohta|first6=K.|last7=Seki|first7=H.|last8=Igarashi|first8=N.|last9=Maruhashi|first9=K.|date=2001-09-15|title=Differential cellular targets of Epstein-Barr virus (EBV) infection between acute EBV-associated hemophagocytic lymphohistiocytosis and chronic active EBV infection|url=https://pubmed.ncbi.nlm.nih.gov/11535525|journal=Blood|volume=98|issue=6|pages=1882–1888|doi=10.1182/blood.v98.6.1882|issn=0006-4971|pmid=11535525}}</ref><ref>{{Cite journal|last=Au|first=W.-Y.|last2=Ma|first2=S.-Y.|last3=Chim|first3=C.-S.|last4=Choy|first4=C.|last5=Loong|first5=F.|last6=Lie|first6=A. K. W.|last7=Lam|first7=C. C. K.|last8=Leung|first8=A. Y. H.|last9=Tse|first9=E.|date=2005-02|title=Clinicopathologic features and treatment outcome of mature T-cell and natural killer-cell lymphomas diagnosed according to the World Health Organization classification scheme: a single center experience of 10 years|url=https://pubmed.ncbi.nlm.nih.gov/15668271|journal=Annals of Oncology: Official Journal of the European Society for Medical Oncology|volume=16|issue=2|pages=206–214|doi=10.1093/annonc/mdi037|issn=0923-7534|pmid=15668271}}</ref><ref name=":9">{{Cite journal|last=Smith|first=Megan C.|last2=Cohen|first2=Daniel N.|last3=Greig|first3=Bruce|last4=Yenamandra|first4=Ashwini|last5=Vnencak-Jones|first5=Cindy|last6=Thompson|first6=Mary Ann|last7=Kim|first7=Annette S.|date=2014|title=The ambiguous boundary between EBV-related hemophagocytic lymphohistiocytosis and systemic EBV-driven T cell lymphoproliferative disorder|url=https://pubmed.ncbi.nlm.nih.gov/25337215|journal=International Journal of Clinical and Experimental Pathology|volume=7|issue=9|pages=5738–5749|issn=1936-2625|pmc=4203186|pmid=25337215}}</ref><ref>{{Cite journal|last=Chen|first=J. S.|last2=Tzeng|first2=C. C.|last3=Tsao|first3=C. J.|last4=Su|first4=W. C.|last5=Chen|first5=T. Y.|last6=Jung|first6=Y. C.|last7=Su|first7=I. J.|date=1997-09|title=Clonal karyotype abnormalities in EBV-associated hemophagocytic syndrome|url=https://pubmed.ncbi.nlm.nih.gov/9407723|journal=Haematologica|volume=82|issue=5|pages=572–576|issn=0390-6078|pmid=9407723}}</ref><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
----
Line 446: Line 313:
|}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.
|}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.


<blockquote class='blockedit'>{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}</blockquote>
<blockquote class="blockedit">{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}</blockquote>




Line 452: Line 319:




<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":4" /><ref name=":8" /><blockquote class="blockedit">
<blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":1" /><ref name=":4" /><ref name=":8" /><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
----
Line 489: Line 356:
|}
|}


<blockquote class='blockedit'>{{Box-round|title=v4:Genes and Main Pathways Involved|The content below was from the old template. Please incorporate above.}}</blockquote>
<blockquote class="blockedit">{{Box-round|title=v4:Genes and Main Pathways Involved|The content below was from the old template. Please incorporate above.}}</blockquote>


*N/A
*N/A
Line 532: Line 399:
(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 />
(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 />


'''
<br />


==Notes==
==Notes==
Line 541: Line 408:
          
          
<nowiki>*</nowiki>''Citation of this Page'': “Systemic EBV-positive T-cell lymphoma of childhood”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Systemic_EBV-positive_T-cell_lymphoma_of_childhood</nowiki>.
<nowiki>*</nowiki>''Citation of this Page'': “Systemic EBV-positive T-cell lymphoma of childhood”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Systemic_EBV-positive_T-cell_lymphoma_of_childhood</nowiki>.
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases S]]
[[Category:HAEM5]]
[[Category:DISEASE]]
[[Category:Diseases S]]