Bailey.Glen: Created page with "==Primary Author(s)*== Fei Yang, MD, FACMG
Oregon Health & Science University, Portland, OR TOC ==Cancer Category/Type== Acute Myeloid Leukemia ==Cancer S..."

2023-11-03T17:34:45Z

Created page with "==Primary Author(s)*== Fei Yang, MD, FACMG <br> Oregon Health & Science University, Portland, OR __TOC__ ==Cancer Category/Type== Acute Myeloid Leukemia ==Cancer S..."

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==Primary Author(s)*==
Fei Yang, MD, FACMG
<br>
Oregon Health & Science University, Portland, OR

__TOC__

==Cancer Category/Type==

[[AML|Acute Myeloid Leukemia]]

==Cancer Sub-Classification / Subtype==

Acute myelomonocytic leukemia

==Definition / Description of Disease==

Acute myelomonocytic leukemia is an acute leukemia characterized by the proliferation of both neutrophil and monocyte precursors. The peripheral blood or bone marrow has more than 20% blasts (including promonocytes). A minimum of 20% monocytes and their precursors is required for the morphological diagnosis, differentiating this entity from cases of AML with or without maturation that can present with a low-level of monocytes. In the 2016 revision to the World Health Organization (WHO) classification system, acute myelomonocytic leukemia is a distinct entity within the section of [[Acute Myeloid Leukemia (AML), Not Otherwise Specified]]<ref>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. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. IARC Press: Lyon, France, p159-160.</ref><ref>{{Cite journal|last=Arber|first=Daniel A.|last2=Orazi|first2=Attilio|last3=Hasserjian|first3=Robert|last4=Thiele|first4=Jürgen|last5=Borowitz|first5=Michael J.|last6=Le Beau|first6=Michelle M.|last7=Bloomfield|first7=Clara D.|last8=Cazzola|first8=Mario|last9=Vardiman|first9=James W.|date=2016|title=The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia|url=https://www.ncbi.nlm.nih.gov/pubmed/27069254|journal=Blood|volume=127|issue=20|pages=2391–2405|doi=10.1182/blood-2016-03-643544|issn=1528-0020|pmid=27069254}}</ref>. This entity does ''not'' meet the criteria for inclusion in any of the other AML groups (i.e. AML with Recurrent Genetic Abnormalities, AML with Myelodysplasia-Related Changes, or Therapy-Related Myeloid Neoplasms).

==Synonyms / Terminology==

French-American-Brirish (FAB) classification M4, NOS

==Epidemiology / Prevalence==

Approximately 5-10% of AML cases, 3% of childhood leukemia

*occurs in all age groups, but is more common in older individuals.
*median patient age is 50 years
*male-to-female ratio is 1.4:1

==Clinical Features==

The common clinical presentations are related to anaemia and thrombocytopenia, including fever, pallor, dyspnea, fatigue, loss of weight and bleeding disorders.

==Sites of Involvement==

Bone marrow

==Morphologic Features==

*Monoblasts are large cells with abundant cytoplasm, which can be moderately to intensely basophillic, and round nuclei with delicate lacy chromatin and one or more predominant nucleoli; scattered azurophillic granuoles, vacuoles, and Auer rods may be present.
*Promonocytes have a more irregular and delicately convoluted nuclear configuration and cytoplasm which is usually less basophillic and more granulated.
*The peripheral blood typically shows an increase in monocytes, which are often more mature than those in the bone marrow.

==Immunophenotype==
Cytochemistry

*≥3% of blasts show myeloperoxidase (MPO) activity.
*Monoblasts, promonocytes and monocytes usually show non-specific esterase activity.

Often a complex immunophenotype with multiple blast populations seen including:

*immature blasts with high CD34 and/or KIT (CD117) expression
*populations with myeloid markers: CD13, CD33, CD15, CD65 and MPO
*populations with monocytic markers: CD4, CD11b, CD11c, CD14, CD64, CD36, CD68 (PGM1), CD163 and lysozyme
*most cases are positive for HLA-DR
*approximately 30% of cases are positive for CD7

{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||EXAMPLE CD1
|-
|Positive (subset)||EXAMPLE CD2
|-
|Negative (universal)||EXAMPLE CD3
|-
|Negative (subset)||EXAMPLE CD4
|}

==Chromosomal Rearrangements (Gene Fusions)==

*None
*if inv(16)(p13.1q22) or t(16;16)(p13.1;q22) are present, classify as [[Acute Myeloid Leukemia (AML) with Recurrent Genetic Abnormalities]]: [[Acute Myeloid Leukemia (AML) with inv(16)(p13.1q22) or t(16;16)(p13.1;q22); CBFB-MYH11]].

==Characteristic Chromosomal Aberrations / Patterns==

Myeloid-associated nonspecific cytogenetic abnormalities, such as trisomy 8, are present in most cases.

==Genomic Gain/Loss/LOH==
None

==Gene Mutations (SNV/INDEL)==

Acute myelomonocytic leukemia has genetic heterogeneity at the molecular level. Currently there is no specific gene identified that is frequently associated with this subset of AML.

{| class="wikitable sortable"
|-
!Gene!!Mutation!!Oncogene/Tumor Suppressor/Other!!Presumed Mechanism (LOF/GOF/Other; Driver/Passenger)!!Prevalence (COSMIC/TCGA/Other)
|-
|EXAMPLE TP53||EXAMPLE R273H||EXAMPLE Tumor Suppressor||EXAMPLE LOF||EXAMPLE 20%
|}

===Other Mutations===
{| class="wikitable sortable"
|-
!Type!!Gene/Region/Other
|-
|Concomitant Mutations||EXAMPLE IDH1 R123H
|-
|Secondary Mutations||EXAMPLE Trisomy 7
|-
|Mutually Exclusive||EXAMPLE EGFR Amplification
|}

==Epigenomics (Methylation)==

None

==Genes and Main Pathways Involved==

None

==Diagnostic Testing Methods==

*Conventional chromosome analysis
*FISH myeloid panel

==Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications)==

Put your text here

==Familial Forms==

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==Other Information==

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==Links==

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==References==
<references />

==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:Acute Myelomonocytic Leukemia - Revision history

Bailey.Glen: Created page with "==Primary Author(s)*== Fei Yang, MD, FACMG Oregon Health & Science University, Portland, OR __TOC__ ==Cancer Category/Type== Acute Myeloid Leukemia ==Cancer S..."

Bailey.Glen: Created page with "==Primary Author(s)*== Fei Yang, MD, FACMG
Oregon Health & Science University, Portland, OR TOC ==Cancer Category/Type== Acute Myeloid Leukemia ==Cancer S..."