Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Fei Yang, MD, FACMG, Oregon Health & Science University TOC ==Cancer Category/Type== [https://ccga.io/index.php/T-Lymphob..."

2023-11-03T17:38:55Z

Created page with "{{Under Construction}} ==Primary Author(s)*== Fei Yang, MD, FACMG, Oregon Health & Science University __TOC__ ==Cancer Category/Type== [https://ccga.io/index.php/T-Lymphob..."

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{{Under Construction}}
==Primary Author(s)*==

Fei Yang, MD, FACMG, Oregon Health & Science University

__TOC__

==Cancer Category/Type==

[https://ccga.io/index.php/T-Lymphoblastic_Leukemia/Lymphoma T-Lymphoblastic Leukemia (T-ALL)]

==Cancer Sub-Classification / Subtype==

NK-Lymphoblastic Leukemia/Lymphoma

==Definition / Description of Disease==

Rare cases of lymphoblastic leukemia/lymphoma express antigens present on NK cells and lack expression of specific markers of T cells, myeloid cells, or plasmacytoid dendritic cells. NK-lymphoblastic leukemia/lymphoma (NK-ALL/LBL) has been very difficult to define, and may be considered in a case that expresses CD56 along with immature T-associated markers such as CD7 and CD2, and even cytoplasmic CD3 in the context of of lacking B-cell and myeloid markers <ref>Borowitz MJ, et al., (2016).T-lymphoblastic leukemia/lymphoma, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4thedition.Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, Editors. IARC Press: Lyon, France, p176-178.</ref>. NK-ALL/LBL is considered as a provisional entity in the current 2016 World Health Organization (WHO) classification system.

==Synonyms / Terminology==

N/A

==Epidemiology / Prevalence==

The prevalence of NK-Lymphoblastic Leukemia/Lymphoma is not well defined. It is a rare and heterogeneous group of immature disorders suggested to be of precursor NK cell origin.

==Clinical Features==

NK-ALL/LBL patients presented with higher white blood cell and platelet counts compared to other CD56+ hematological disorders <ref name=":0">{{Cite journal|last=Weinberg|first=Olga K.|last2=Chisholm|first2=Karen M.|last3=Ok|first3=Chi Young|last4=Fedoriw|first4=Yuri|last5=Grzywacz|first5=Bartosz|last6=Kurzer|first6=Jason H.|last7=Mason|first7=Emily F.|last8=Moser|first8=Karen A.|last9=Bhattacharya|first9=Siddharth|date=2021-07|title=Clinical, immunophenotypic and genomic findings of NK lymphoblastic leukemia: a study from the Bone Marrow Pathology Group|url=https://www.nature.com/articles/s41379-021-00739-4|journal=Modern Pathology|language=en|volume=34|issue=7|pages=1358–1366|doi=10.1038/s41379-021-00739-4|issn=1530-0285}}</ref>.
==Sites of Involvement==

Bone marrow, lymph node, and extra nodal lesion.

==Morphologic Features==

In an early report, the morphologic features were described as immature mononuclear cells intermediate to large in size, with variable cytoplasm and vesicular chromatin <ref>{{Cite journal|last=Sedick|first=Qanita|last2=Alotaibi|first2=Sultan|last3=Alshieban|first3=Saeed|last4=Naheet|first4=Khalid Ben|last5=Elyamany|first5=Ghaleb|date=2017-05|title=Natural Killer Cell Lymphoblastic Leukaemia/Lymphoma: Case Report and Review of the Recent Literature|url=https://pubmed.ncbi.nlm.nih.gov/28868017|journal=Case Reports in Oncology|volume=10|issue=2|pages=588–595|doi=10.1159/000477843|issn=1662-6575|pmc=5567073|pmid=28868017}}</ref>.

==Immunophenotype==

<br />

{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||CD56, CD34, CD2, CD7
|-
|Positive (subset)||CD33, cCD3, CD5, CD16, CD94
|-
|Negative (universal)||N/A
|-
|Negative (subset)||N/A
|}

==Chromosomal Rearrangements (Gene Fusions)==

Chromosomal rearrangement abnormalities of NK-ALL/LBL are not well defined. The assessment of TCR gene rearrangement is usually negative.
==Individual Region Genomic Gain/Loss/LOH==

Copy number alterations/Loss of heterozygosity of NK-ALL/LBL are not well defined.
==Characteristic Chromosomal Patterns==

Chromosomal abnormalities of NK-ALL/LBL are not well defined. In a recent multi-institutional study, complex karyotype has been reported in more than 50% of the NK-ALL/LBL patients <ref name=":0" />.
==Gene Mutations (SNV/INDEL)==

Genetic abnormalities of NK-ALL/LBL are not well defined. In a recent multi-institutional study, pathogenic mutations were reported common in NOTCH1, ETV6, and JAK3 genes <ref name=":0" />.

Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.

==Epigenomic Alterations==

N/A

==Genes and Main Pathways Involved==

<br />
{| class="wikitable sortable"
|-
!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome
|-
|ETV6; Inactivating mutations
|Transcriptional repressor
|Increased cell growth and proliferation
|-
|NOTCH1; Activating mutations
|Signaling transduction
|Increased cell differentiation, growth and proliferation
|}<br />
==Diagnostic Testing Methods==

Clinical, morphological, and immunophenotypic findings are generally sufficient for diagnosis.

==Familial Forms==

Unknown

==Additional Information==

N/A

==Links==

N/A

==References==
<references />
(use "Cite" icon at top of page)
===EXAMPLE Book===

#Arber DA, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p129-171.

==Notes==
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.

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HAEM4Backup:NK-Lymphoblastic Leukemia/Lymphoma - Revision history

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Fei Yang, MD, FACMG, Oregon Health & Science University __TOC__ ==Cancer Category/Type== [https://ccga.io/index.php/T-Lymphob..."

Bailey.Glen: Created page with "{{Under Construction}} ==Primary Author(s)*== Fei Yang, MD, FACMG, Oregon Health & Science University TOC ==Cancer Category/Type== [https://ccga.io/index.php/T-Lymphob..."