Changes

==Definition / Description of Disease==

==Definition / Description of Disease==

Acute megakaryoblastic leukemia is a myeloid disease defined by ≥20% blasts in the peripheral blood or bone marrow, of which ≥50% are of megakaryocyte lineage. In the 2016 revision to the World Health Organization (WHO) classification system, acute megakaryoblastic 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, p162-164.</ref><ref name=":0">{{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).  

Acute megakaryoblastic leukemia is a myeloid disease defined by ≥20% blasts in the peripheral blood or bone marrow, of which ≥50% are of megakaryocyte lineage. In the 2016 revision to the World Health Organization (WHO) classification system, acute megakaryoblastic leukemia is a distinct entity within the section of [[HAEM4: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, p162-164.</ref><ref name=":0">{{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).  

AMKL in an individual with Down syndrome should be classified as a different entity, specifically [[Myeloid Leukemia Associated with Down Syndrome]]<ref name=":0" />.

AMKL in an individual with Down syndrome should be classified as a different entity, specifically [[HAEM5:Myeloid proliferations associated with Down syndrome]]<ref name=":0" />.

AMKL associated with t(1;22)(p13.3;q13.1), or inv(3)(q21.3q26.2)/t(3;3)(q21.3;q26.2) should be classified as a different entity, specifically [[Acute Myeloid Leukemia (AML) with Recurrent Genetic Abnormalities]]: [[Acute Myeloid Leukemia (AML) Megakaryoblastic with t(1;22)(p13.3;q13.1);RBM15-MKL1]] or [[Acute Myeloid Leukemia (AML) with inv(3)(q21.3q26.2) or t(3;3)(q21.3;q26.2);GATA2, MECOM]]<ref name=":0" />.

AMKL associated with t(1;22)(p13.3;q13.1), or inv(3)(q21.3q26.2)/t(3;3)(q21.3;q26.2) should be classified as a different entity, specifically [[HAEM4:Acute Myeloid Leukemia (AML) with Recurrent Genetic Abnormalities]]: [[HAEM5:Acute myeloid leukaemia with RBM15::MRTFA fusion]] or [[HAEM5:Acute myeloid leukaemia with MECOM rearrangement]]<ref name=":0" />.

==Synonyms / Terminology==

==Synonyms / Terminology==

Prognosis

Prognosis

*The prognosis of AMKL is usually poorer than that of other AML types,  [[Acute Myeloid Leukemia (AML) Megakaryoblastic with t(1;22)(p13.3;q13.1);RBM15-MKL1]], and [[ Myeloid Leukemia Associated with Down Syndrome]]<ref name=":0" /><ref>{{Cite journal|last=Oki|first=Yasuhiro|last2=Kantarjian|first2=Hagop M.|last3=Zhou|first3=Xian|last4=Cortes|first4=Jorge|last5=Faderl|first5=Stefan|last6=Verstovsek|first6=Srdan|last7=O'Brien|first7=Susan|last8=Koller|first8=Charles|last9=Beran|first9=Miloslav|date=2006|title=Adult acute megakaryocytic leukemia: an analysis of 37 patients treated at M.D. Anderson Cancer Center|url=https://www.ncbi.nlm.nih.gov/pubmed/16123215|journal=Blood|volume=107|issue=3|pages=880–884|doi=10.1182/blood-2005-06-2450|issn=0006-4971|pmid=16123215}}</ref>.

*The prognosis of AMKL is usually poorer than that of other AML types,  [[HAEM5:Acute myeloid leukaemia with RBM15::MRTFA fusion]], and [[HAEM5:Myeloid proliferations associated with Down syndrome]]<ref name=":0" /><ref>{{Cite journal|last=Oki|first=Yasuhiro|last2=Kantarjian|first2=Hagop M.|last3=Zhou|first3=Xian|last4=Cortes|first4=Jorge|last5=Faderl|first5=Stefan|last6=Verstovsek|first6=Srdan|last7=O'Brien|first7=Susan|last8=Koller|first8=Charles|last9=Beran|first9=Miloslav|date=2006|title=Adult acute megakaryocytic leukemia: an analysis of 37 patients treated at M.D. Anderson Cancer Center|url=https://www.ncbi.nlm.nih.gov/pubmed/16123215|journal=Blood|volume=107|issue=3|pages=880–884|doi=10.1182/blood-2005-06-2450|issn=0006-4971|pmid=16123215}}</ref>.

==Familial Forms==

==Familial Forms==