Compendium of Cancer Genome Aberrations

HAEM5:Extranodal NK/T-cell lymphoma: Difference between revisions

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<nowiki>***</nowiki>Amanda, we do not have the revised 4th edition in our resident office... could you please check the page numbers and update the reference with the correct author and page numbers? thanks!
<nowiki>***</nowiki>'''Amanda, we do not have the revised 4th edition in our resident office... could you please check the page numbers and update the reference with the correct author and page numbers? thanks!'''


Extranodal NK/T-cell lymphoma (ENKTL) is a lymphoma of NK or T-cell lineage strongly associated with Epstein-Barr virus (citation needed)


ENKTL is a destructive angiocentric disease characterized by vascular destruction and necrosis (citation needed)
It can be clinically divided into nasal and non-nasal types (citation needed)


Put your text here <span style="color:#0070C0">(''Instructions: Brief description of approximately one paragraph - include disease context relative to other WHO classification categories referring to the specific WHO book pages, diagnostic criteria if applicable, and differential diagnosis if applicable'') </span>
Put your text here <span style="color:#0070C0">(''Instructions: Brief description of approximately one paragraph - include disease context relative to other WHO classification categories referring to the specific WHO book pages, diagnostic criteria if applicable, and differential diagnosis if applicable'') </span>
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==Synonyms / Terminology==
==Synonyms / Terminology==


Extranodal NK/T-cell lymphoma, nasal type; EBV-positive extranodal NK/T-cell lymphoma; angiocentric lymphoma (historical); lethal midline granuloma (historical)
Extranodal NK/T-cell lymphoma, nasal type; EBV-positive extranodal NK/T-cell lymphoma; angiocentric lymphoma (not recommended); lethal midline granuloma (historical)


==Epidemiology / Prevalence==
==Epidemiology / Prevalence==


Put your text here
ENKTL is most prevalent in East Asia and Latin America. It represents less than 1% of non-Hodgkin lymphomas in the United States, with the highest incidence among Asian Pacific Islanders and Hispanic populations<ref>{{Cite journal|last=Haverkos|first=Bradley M.|last2=Pan|first2=Zenggang|last3=Gru|first3=Alejandro A.|last4=Freud|first4=Aharon G.|last5=Rabinovitch|first5=Rachel|last6=Xu-Welliver|first6=Meng|last7=Otto|first7=Brad|last8=Barrionuevo|first8=Carlos|last9=Baiocchi|first9=Robert A.|date=2016-12|title=Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT): An update on epidemiology, clinical presentation, and natural history in North American and European cases|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5199232/|journal=Current hematologic malignancy reports|volume=11|issue=6|pages=514–527|doi=10.1007/s11899-016-0355-9|issn=1558-8211|pmc=5199232|pmid=27778143}}</ref>.


==Clinical Features==
==Clinical Features==


Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table'') </span>
Common clinical presentations of nasal-type ENKTL include nasal mass, obstruction, and bleeding. Extranasal ENKTL may involve the skin, testis, and gastrointestinal tract<ref>Thida AM, Gohari P. Extranodal NK-Cell Lymphoma. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: <nowiki>https://www.ncbi.nlm.nih.gov/books/NBK559207/</nowiki></ref>. The presence of B symptoms is associated with higher clinical stage<ref>{{Cite journal|last=Takahara|first=Miki|last2=Kumai|first2=Takumi|last3=Kishibe|first3=Kan|last4=Nagato|first4=Toshihiro|last5=Harabuchi|first5=Yasuaki|date=2021-06-25|title=Extranodal NK/T-Cell Lymphoma, Nasal Type: Genetic, Biologic, and Clinical Aspects with a Central Focus on Epstein-Barr Virus Relation|url=https://pubmed.ncbi.nlm.nih.gov/34202088|journal=Microorganisms|volume=9|issue=7|pages=1381|doi=10.3390/microorganisms9071381|issn=2076-2607|pmc=8304202|pmid=34202088}}</ref>.
{| class="wikitable"
{| class="wikitable"
|'''Signs and Symptoms'''
|'''Signs and Symptoms'''
|EXAMPLE Asymptomatic (incidental finding on complete blood counts)
|Nasal mass, nasal obstruction, nasal bleeding
Hoarseness, dysphagia, halitosis, airway obstruction, dysphonia


EXAMPLE B-symptoms (weight loss, fever, night sweats)
Abdominal pain, GI bleeding, bowel perforation


EXAMPLE Fatigue
B symptoms (fever, weight loss, night sweats)
 
EXAMPLE Lymphadenopathy (uncommon)
|-
|-
|'''Laboratory Findings'''
|'''Laboratory Findings'''
|EXAMPLE Cytopenias
|No specific findings
 
Cytopenias
EXAMPLE Lymphocytosis (low level)
|}
|}


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