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{{Acute megakaryoblastic leukemia}}
{{Acute megakaryoblastic leukemia}}


*AMKL is the most common subtype of AML reported in Down syndrome patients.<ref name="XavierGe2009">{{cite journal|last1=Xavier|first1=Ana C.|last2=Ge|first2=Yubin|last3=Taub|first3=Jeffrey W.|title=Down Syndrome and Malignancies: A Unique Clinical Relationship|journal=The Journal of Molecular Diagnostics|volume=11|issue=5|year=2009|pages=371–380|issn=15251578|doi=10.2353/jmoldx.2009.080132}}</ref> It is more common in children compared with adults. The prevalence in children and adults is ~15% and 0.6%, respectively.<ref name="XavierGe20092">{{cite journal|last1=Xavier|first1=Ana C.|last2=Ge|first2=Yubin|last3=Taub|first3=Jeffrey W.|title=Down Syndrome and Malignancies: A Unique Clinical Relationship|journal=The Journal of Molecular Diagnostics|volume=11|issue=5|year=2009|pages=371–380|issn=15251578|doi=10.2353/jmoldx.2009.080132}}</ref><ref name="PaganoPulsoni2002">{{cite journal|last1=Pagano|first1=L|last2=Pulsoni|first2=A|last3=Vignetti|first3=M|last4=Mele|first4=L|last5=Fianchi|first5=L|last6=Petti|first6=MC|last7=Mirto|first7=S|last8=Falcucci|first8=P|last9=Fazi|first9=P|last10=Broccia|first10=G|last11=Specchia|first11=G|last12=Di Raimondo|first12=F|last13=Pacilli|first13=L|last14=Leoni|first14=P|last15=Ladogana|first15=S|last16=Gallo|first16=E|last17=Venditti|first17=A|last18=Avanzi|first18=G|last19=Camera|first19=A|last20=Liso|first20=V|last21=Leone|first21=G|last22=Mandelli|first22=F|title=Acute megakaryoblastic leukemia: experience of GIMEMA trials|journal=Leukemia|volume=16|issue=9|year=2002|pages=1622–1626|issn=0887-6924|doi=10.1038/sj.leu.2402618}}</ref>
{{CMG}}; {{AE}}
==Overview==
 
==Epidemiology and Demographics==
 
* AMKL is the most common subtype of AML reported in [[Down syndrome]] patients.<ref name="XavierGe2009">{{cite journal|last1=Xavier|first1=Ana C.|last2=Ge|first2=Yubin|last3=Taub|first3=Jeffrey W.|title=Down Syndrome and Malignancies: A Unique Clinical Relationship|journal=The Journal of Molecular Diagnostics|volume=11|issue=5|year=2009|pages=371–380|issn=15251578|doi=10.2353/jmoldx.2009.080132}}</ref> It is more common in children compared with adults. The [[prevalence]] in children and adults is ~15% and 0.6%, respectively.<ref name="XavierGe20092">{{cite journal|last1=Xavier|first1=Ana C.|last2=Ge|first2=Yubin|last3=Taub|first3=Jeffrey W.|title=Down Syndrome and Malignancies: A Unique Clinical Relationship|journal=The Journal of Molecular Diagnostics|volume=11|issue=5|year=2009|pages=371–380|issn=15251578|doi=10.2353/jmoldx.2009.080132}}</ref><ref name="PaganoPulsoni2002">{{cite journal|last1=Pagano|first1=L|last2=Pulsoni|first2=A|last3=Vignetti|first3=M|last4=Mele|first4=L|last5=Fianchi|first5=L|last6=Petti|first6=MC|last7=Mirto|first7=S|last8=Falcucci|first8=P|last9=Fazi|first9=P|last10=Broccia|first10=G|last11=Specchia|first11=G|last12=Di Raimondo|first12=F|last13=Pacilli|first13=L|last14=Leoni|first14=P|last15=Ladogana|first15=S|last16=Gallo|first16=E|last17=Venditti|first17=A|last18=Avanzi|first18=G|last19=Camera|first19=A|last20=Liso|first20=V|last21=Leone|first21=G|last22=Mandelli|first22=F|title=Acute megakaryoblastic leukemia: experience of GIMEMA trials|journal=Leukemia|volume=16|issue=9|year=2002|pages=1622–1626|issn=0887-6924|doi=10.1038/sj.leu.2402618}}</ref>
 
*Down syndrome patients carry a 200 fold increased risk of developing AMKL vs. non-Down syndrome patients.<ref name="ZipurskyPeeters20092">{{cite journal|last1=Zipursky|first1=Alvin|last2=Peeters|first2=Marie|last3=Poon|first3=Annette|title=Megakaryoblastic Leukemia and Down's Syndrome: A Review|journal=Pediatric Hematology and Oncology|volume=4|issue=3|year=2009|pages=211–230|issn=0888-0018|doi=10.3109/08880018709141272}}</ref> Similarly, another review proposed a 500 fold increased risk.<ref name="AthaleRazzouk2001">{{cite journal|last1=Athale|first1=Uma H.|last2=Razzouk|first2=Bassem I.|last3=Raimondi|first3=Susana C.|last4=Tong|first4=Xin|last5=Behm|first5=Frederick G.|last6=Head|first6=David R.|last7=Srivastava|first7=Deo K.|last8=Rubnitz|first8=Jeffrey E.|last9=Bowman|first9=Laura|last10=Pui|first10=Ching-Hon|last11=Ribeiro|first11=Raul C.|title=Biology and outcome of childhood acute megakaryoblastic leukemia: a single institution's experience|journal=Blood|volume=97|issue=12|year=2001|pages=3727–3732|issn=1528-0020|doi=10.1182/blood.V97.12.3727}}</ref> It almost exclusively occurs in the first 3 years of life.<ref name="XavierGe20094">{{cite journal|last1=Xavier|first1=Ana C.|last2=Ge|first2=Yubin|last3=Taub|first3=Jeffrey W.|title=Down Syndrome and Malignancies: A Unique Clinical Relationship|journal=The Journal of Molecular Diagnostics|volume=11|issue=5|year=2009|pages=371–380|issn=15251578|doi=10.2353/jmoldx.2009.080132}}</ref><ref name="ZipurskyPeeters20094">{{cite journal|last1=Zipursky|first1=Alvin|last2=Peeters|first2=Marie|last3=Poon|first3=Annette|title=Megakaryoblastic Leukemia and Down's Syndrome: A Review|journal=Pediatric Hematology and Oncology|volume=4|issue=3|year=2009|pages=211–230|issn=0888-0018|doi=10.3109/08880018709141272}}</ref>
*Down syndrome patients carry a 200 fold increased risk of developing AMKL vs. non-Down syndrome patients.<ref name="ZipurskyPeeters20092">{{cite journal|last1=Zipursky|first1=Alvin|last2=Peeters|first2=Marie|last3=Poon|first3=Annette|title=Megakaryoblastic Leukemia and Down's Syndrome: A Review|journal=Pediatric Hematology and Oncology|volume=4|issue=3|year=2009|pages=211–230|issn=0888-0018|doi=10.3109/08880018709141272}}</ref> Similarly, another review proposed a 500 fold increased risk.<ref name="AthaleRazzouk2001">{{cite journal|last1=Athale|first1=Uma H.|last2=Razzouk|first2=Bassem I.|last3=Raimondi|first3=Susana C.|last4=Tong|first4=Xin|last5=Behm|first5=Frederick G.|last6=Head|first6=David R.|last7=Srivastava|first7=Deo K.|last8=Rubnitz|first8=Jeffrey E.|last9=Bowman|first9=Laura|last10=Pui|first10=Ching-Hon|last11=Ribeiro|first11=Raul C.|title=Biology and outcome of childhood acute megakaryoblastic leukemia: a single institution's experience|journal=Blood|volume=97|issue=12|year=2001|pages=3727–3732|issn=1528-0020|doi=10.1182/blood.V97.12.3727}}</ref> It almost exclusively occurs in the first 3 years of life.<ref name="XavierGe20094">{{cite journal|last1=Xavier|first1=Ana C.|last2=Ge|first2=Yubin|last3=Taub|first3=Jeffrey W.|title=Down Syndrome and Malignancies: A Unique Clinical Relationship|journal=The Journal of Molecular Diagnostics|volume=11|issue=5|year=2009|pages=371–380|issn=15251578|doi=10.2353/jmoldx.2009.080132}}</ref><ref name="ZipurskyPeeters20094">{{cite journal|last1=Zipursky|first1=Alvin|last2=Peeters|first2=Marie|last3=Poon|first3=Annette|title=Megakaryoblastic Leukemia and Down's Syndrome: A Review|journal=Pediatric Hematology and Oncology|volume=4|issue=3|year=2009|pages=211–230|issn=0888-0018|doi=10.3109/08880018709141272}}</ref>
*Transient leukemia (TL) occurs in approximately 10% of Down syndrome infants, which is also attributed to transient myeloproliferative disorder.<ref name="Zipursky2003">{{cite journal|last1=Zipursky|first1=Alvin|title=Transient leukaemia - a benign form of leukaemia in newborn infants with trisomy 21|journal=British Journal of Haematology|volume=120|issue=6|year=2003|pages=930–938|issn=00071048|doi=10.1046/j.1365-2141.2003.04229.x}}</ref><ref name="PineGuo2007">{{cite journal|last1=Pine|first1=Sharon R.|last2=Guo|first2=Qianxu|last3=Yin|first3=Changhong|last4=Jayabose|first4=Somasundaram|last5=Druschel|first5=Charlotte M.|last6=Sandoval|first6=Claudio|title=Incidence and clinical implications of GATA1 mutations in newborns with Down syndrome|journal=Blood|volume=110|issue=6|year=2007|pages=2128–2131|issn=0006-4971|doi=10.1182/blood-2007-01-069542}}</ref> In most cases, TL spontaneously resolves; however, during the first four years of life, it progresses to acute megakaryoblastic leukemia in 13% to 33% of patients.<ref name="KlusmannCreutzig2008">{{cite journal|last1=Klusmann|first1=Jan-Henning|last2=Creutzig|first2=Ursula|last3=Zimmermann|first3=Martin|last4=Dworzak|first4=Michael|last5=Jorch|first5=Norbert|last6=Langebrake|first6=Claudia|last7=Pekrun|first7=Arnulf|last8=Macakova-Reinhardt|first8=Katarina|last9=Reinhardt|first9=Dirk|title=Treatment and prognostic impact of transient leukemia in neonates with Down syndrome|journal=Blood|volume=111|issue=6|year=2008|pages=2991–2998|issn=0006-4971|doi=10.1182/blood-2007-10-118810}}</ref>
*[[Transient leukemia]] (TL) occurs in approximately 10% of Down syndrome infants, which is also attributed to transient [[myeloproliferative disorder]].<ref name="Zipursky2003">{{cite journal|last1=Zipursky|first1=Alvin|title=Transient leukaemia - a benign form of leukaemia in newborn infants with trisomy 21|journal=British Journal of Haematology|volume=120|issue=6|year=2003|pages=930–938|issn=00071048|doi=10.1046/j.1365-2141.2003.04229.x}}</ref><ref name="PineGuo2007">{{cite journal|last1=Pine|first1=Sharon R.|last2=Guo|first2=Qianxu|last3=Yin|first3=Changhong|last4=Jayabose|first4=Somasundaram|last5=Druschel|first5=Charlotte M.|last6=Sandoval|first6=Claudio|title=Incidence and clinical implications of GATA1 mutations in newborns with Down syndrome|journal=Blood|volume=110|issue=6|year=2007|pages=2128–2131|issn=0006-4971|doi=10.1182/blood-2007-01-069542}}</ref> In most cases, TL spontaneously resolves; however, during the first four years of life, it progresses to acute megakaryoblastic leukemia in 13% to 33% of patients.<ref name="KlusmannCreutzig2008">{{cite journal|last1=Klusmann|first1=Jan-Henning|last2=Creutzig|first2=Ursula|last3=Zimmermann|first3=Martin|last4=Dworzak|first4=Michael|last5=Jorch|first5=Norbert|last6=Langebrake|first6=Claudia|last7=Pekrun|first7=Arnulf|last8=Macakova-Reinhardt|first8=Katarina|last9=Reinhardt|first9=Dirk|title=Treatment and prognostic impact of transient leukemia in neonates with Down syndrome|journal=Blood|volume=111|issue=6|year=2008|pages=2991–2998|issn=0006-4971|doi=10.1182/blood-2007-10-118810}}</ref>


==References==
==References==

Latest revision as of 14:09, 26 April 2021

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Epidemiology and Demographics

  • AMKL is the most common subtype of AML reported in Down syndrome patients.[1] It is more common in children compared with adults. The prevalence in children and adults is ~15% and 0.6%, respectively.[2][3]
  • Down syndrome patients carry a 200 fold increased risk of developing AMKL vs. non-Down syndrome patients.[4] Similarly, another review proposed a 500 fold increased risk.[5] It almost exclusively occurs in the first 3 years of life.[6][7]
  • Transient leukemia (TL) occurs in approximately 10% of Down syndrome infants, which is also attributed to transient myeloproliferative disorder.[8][9] In most cases, TL spontaneously resolves; however, during the first four years of life, it progresses to acute megakaryoblastic leukemia in 13% to 33% of patients.[10]

References

  1. Xavier, Ana C.; Ge, Yubin; Taub, Jeffrey W. (2009). "Down Syndrome and Malignancies: A Unique Clinical Relationship". The Journal of Molecular Diagnostics. 11 (5): 371–380. doi:10.2353/jmoldx.2009.080132. ISSN 1525-1578.
  2. Xavier, Ana C.; Ge, Yubin; Taub, Jeffrey W. (2009). "Down Syndrome and Malignancies: A Unique Clinical Relationship". The Journal of Molecular Diagnostics. 11 (5): 371–380. doi:10.2353/jmoldx.2009.080132. ISSN 1525-1578.
  3. Pagano, L; Pulsoni, A; Vignetti, M; Mele, L; Fianchi, L; Petti, MC; Mirto, S; Falcucci, P; Fazi, P; Broccia, G; Specchia, G; Di Raimondo, F; Pacilli, L; Leoni, P; Ladogana, S; Gallo, E; Venditti, A; Avanzi, G; Camera, A; Liso, V; Leone, G; Mandelli, F (2002). "Acute megakaryoblastic leukemia: experience of GIMEMA trials". Leukemia. 16 (9): 1622–1626. doi:10.1038/sj.leu.2402618. ISSN 0887-6924.
  4. Zipursky, Alvin; Peeters, Marie; Poon, Annette (2009). "Megakaryoblastic Leukemia and Down's Syndrome: A Review". Pediatric Hematology and Oncology. 4 (3): 211–230. doi:10.3109/08880018709141272. ISSN 0888-0018.
  5. Athale, Uma H.; Razzouk, Bassem I.; Raimondi, Susana C.; Tong, Xin; Behm, Frederick G.; Head, David R.; Srivastava, Deo K.; Rubnitz, Jeffrey E.; Bowman, Laura; Pui, Ching-Hon; Ribeiro, Raul C. (2001). "Biology and outcome of childhood acute megakaryoblastic leukemia: a single institution's experience". Blood. 97 (12): 3727–3732. doi:10.1182/blood.V97.12.3727. ISSN 1528-0020.
  6. Xavier, Ana C.; Ge, Yubin; Taub, Jeffrey W. (2009). "Down Syndrome and Malignancies: A Unique Clinical Relationship". The Journal of Molecular Diagnostics. 11 (5): 371–380. doi:10.2353/jmoldx.2009.080132. ISSN 1525-1578.
  7. Zipursky, Alvin; Peeters, Marie; Poon, Annette (2009). "Megakaryoblastic Leukemia and Down's Syndrome: A Review". Pediatric Hematology and Oncology. 4 (3): 211–230. doi:10.3109/08880018709141272. ISSN 0888-0018.
  8. Zipursky, Alvin (2003). "Transient leukaemia - a benign form of leukaemia in newborn infants with trisomy 21". British Journal of Haematology. 120 (6): 930–938. doi:10.1046/j.1365-2141.2003.04229.x. ISSN 0007-1048.
  9. Pine, Sharon R.; Guo, Qianxu; Yin, Changhong; Jayabose, Somasundaram; Druschel, Charlotte M.; Sandoval, Claudio (2007). "Incidence and clinical implications of GATA1 mutations in newborns with Down syndrome". Blood. 110 (6): 2128–2131. doi:10.1182/blood-2007-01-069542. ISSN 0006-4971.
  10. Klusmann, Jan-Henning; Creutzig, Ursula; Zimmermann, Martin; Dworzak, Michael; Jorch, Norbert; Langebrake, Claudia; Pekrun, Arnulf; Macakova-Reinhardt, Katarina; Reinhardt, Dirk (2008). "Treatment and prognostic impact of transient leukemia in neonates with Down syndrome". Blood. 111 (6): 2991–2998. doi:10.1182/blood-2007-10-118810. ISSN 0006-4971.

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