Acute megakaryoblastic leukemia (AMKL) is life-threatening leukemia in which malignant megakaryoblasts proliferate abnormally and injure various tissues. Megakaryoblasts are the most immature precursor cells in a platelet-forming lineage; they mature to promegakaryocytes and, ultimately, megakaryocytes which cells shed membrane-enclosed particles, i.e. platelets, into the circulation. Platelets are critical for the normal clotting of blood. While malignant megakaryoblasts usually are the predominant proliferating and tissue-damaging cells, their similarly malignant descendants, promegakaryocytes and megakaryocytes, are variable contributors to the malignancy.[1]
AMKL is commonly regarded as a subtype of acute myeloid leukemia (AML). More formally, it is classified under the AML-M7 category of the French-American-British classification[2] and by the World Health Organization of 2016 in the AML-Not Otherwise Specified subcategory.[3]
Acute megakaryoblastic leukemia falls into three distinct groups which differ in underlying causes, ages of presentation, responses to therapy, and prognoses. These groups are: AMKL occurring in young children with Down syndrome, i.e. DS-AMKL; AMKL occurring in children who do not have Down syndrome, i.e. non-DS-AMKL (also termed pediatric acute megakaryoblastic leukemia or pediatric AMKL); and AMKL occurring in non-DS adults, i.e. adult-AMKL.[1] AMKL, while rare, is the most common form of AML in DS-AMKL, occurring ~500-fold more commonly in Down syndrome children than in children without Down syndrome; non-DS-AMKL and adult-AMLK are rare, accounting for <1% of all individuals diagnosed as in the AML-M7 category of leukemia.[4]
^ abHahn AW, Li B, Prouet P, Giri S, Pathak R, Martin MG (January 2016). "Acute megakaryocytic leukemia: What have we learned". Blood Reviews. 30 (1): 49–53. doi:10.1016/j.blre.2015.07.005. PMID 26228843.
^"Acute Myeloid Leukemia - Signs and Symptoms".
^Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, Bloomfield CD, Cazzola M, Vardiman JW (May 2016). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–405. doi:10.1182/blood-2016-03-643544. PMID 27069254.
^Seewald L, Taub JW, Maloney KW, McCabe ER (September 2012). "Acute leukemias in children with Down syndrome". Molecular Genetics and Metabolism. 107 (1–2): 25–30. doi:10.1016/j.ymgme.2012.07.011. PMID 22867885.
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Jiang J, et al. (May 2005). "Acute panmyelosis with myelofibrosis: an entity distinct from acutemegakaryoblasticleukemia". Mod. Pathol. 18 (5): 603–14...
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