Changes

==Definition / Description of Disease==

==Definition / Description of Disease==

In the 2008 WHO classification, Acute Erythroid leukemia (AEL) was classified into two subtypes: Erythroleukemia (erythroid/myeloid) and Pure Erythroid Leukemia (PEL). However, in the 2016 WHO update, Erythroleukemia was merged into myelodysplastic syndrome, while PEL is now the only type of AEL<ref name=":0">Arber DA, et al., (2008). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, Editors. IARC Press: Lyon, France, p135-136.</ref><ref name=":1">{{Cite journal|last=Qiu|first=Shaowei|last2=Jiang|first2=Erlie|last3=Wei|first3=Hui|last4=Lin|first4=Dong|last5=Zhang|first5=Guangji|last6=Wei|first6=Shuning|last7=Zhou|first7=Chunlin|last8=Liu|first8=Kaiqi|last9=Wang|first9=Ying|date=2017|title=An analysis of 97 previously diagnosed de novo adult acute erythroid leukemia patients following the 2016 revision to World Health Organization classification|url=https://www.ncbi.nlm.nih.gov/pubmed/28793875|journal=BMC cancer|volume=17|issue=1|pages=534|doi=10.1186/s12885-017-3528-6|issn=1471-2407|pmc=5550989|pmid=28793875}}</ref><ref name=":2">{{Cite journal|last=Zuo|first=Zhuang|last2=Polski|first2=Jacek M.|last3=Kasyan|first3=Armen|last4=Medeiros|first4=L. Jeffrey|date=2010|title=Acute erythroid leukemia|url=https://www.ncbi.nlm.nih.gov/pubmed/20807044|journal=Archives of Pathology & Laboratory Medicine|volume=134|issue=9|pages=1261–1270|doi=10.1043/2009-0350-RA.1|issn=1543-2165|pmid=20807044}}</ref><ref name=":3">{{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><ref name=":4">{{Cite journal|last=Zuo|first=Zhuang|last2=Medeiros|first2=L. Jeffrey|last3=Chen|first3=Zhao|last4=Liu|first4=Dingsheng|last5=Bueso-Ramos|first5=Carlos E.|last6=Luthra|first6=Rajyalakshmi|last7=Wang|first7=Sa A.|date=2012|title=Acute myeloid leukemia (AML) with erythroid predominance exhibits clinical and molecular characteristics that differ from other types of AML|url=https://www.ncbi.nlm.nih.gov/pubmed/22844482|journal=PloS One|volume=7|issue=7|pages=e41485|doi=10.1371/journal.pone.0041485|issn=1932-6203|pmc=3402404|pmid=22844482}}</ref><ref name=":5">{{Cite journal|last=Grossmann|first=V.|last2=Bacher|first2=U.|last3=Haferlach|first3=C.|last4=Schnittger|first4=S.|last5=Pötzinger|first5=F.|last6=Weissmann|first6=S.|last7=Roller|first7=A.|last8=Eder|first8=C.|last9=Fasan|first9=A.|date=2013|title=Acute erythroid leukemia (AEL) can be separated into distinct prognostic subsets based on cytogenetic and molecular genetic characteristics|url=https://www.ncbi.nlm.nih.gov/pubmed/23648669|journal=Leukemia|volume=27|issue=9|pages=1940–1943|doi=10.1038/leu.2013.144|issn=1476-5551|pmid=23648669}}</ref><ref name=":6">{{Cite journal|last=Porwit|first=Anna|last2=Vardiman|first2=James W.|date=2011|title=Acute myeloid leukemia with expanded erythropoiesis|url=https://www.ncbi.nlm.nih.gov/pubmed/21880638|journal=Haematologica|volume=96|issue=9|pages=1241–1243|doi=10.3324/haematol.2011.050526|issn=1592-8721|pmc=3166090|pmid=21880638}}</ref><ref name=":7">{{Cite journal|last=Hasserjian|first=Robert P.|last2=Zuo|first2=Zhuang|last3=Garcia|first3=Christine|last4=Tang|first4=Guilin|last5=Kasyan|first5=Armen|last6=Luthra|first6=Rajyalakshmi|last7=Abruzzo|first7=Lynne V.|last8=Kantarjian|first8=Hagop M.|last9=Medeiros|first9=L. Jeffrey|date=2010|title=Acute erythroid leukemia: a reassessment using criteria refined in the 2008 WHO classification|url=https://www.ncbi.nlm.nih.gov/pubmed/20040759|journal=Blood|volume=115|issue=10|pages=1985–1992|doi=10.1182/blood-2009-09-243964|issn=1528-0020|pmc=2942006|pmid=20040759}}</ref><ref name=":8">{{Cite journal|last=Wang|first=Sa A.|last2=Hasserjian|first2=Robert P.|date=2015|title=Acute Erythroleukemias, Acute Megakaryoblastic Leukemias, and Reactive Mimics: A Guide to a Number of Perplexing Entities|url=https://www.ncbi.nlm.nih.gov/pubmed/26071461|journal=American Journal of Clinical Pathology|volume=144|issue=1|pages=44–60|doi=10.1309/AJCPRKYAT6EZQHC7|issn=1943-7722|pmid=26071461}}</ref><ref name=":9">{{Cite journal|last=Wang|first=Wei|last2=Wang|first2=Sa A.|last3=Medeiros|first3=L. Jeffrey|last4=Khoury|first4=Joseph D.|date=2017|title=Pure erythroid leukemia|url=https://www.ncbi.nlm.nih.gov/pubmed/28006859|journal=American Journal of Hematology|volume=92|issue=3|pages=292–296|doi=10.1002/ajh.24626|issn=1096-8652|pmid=28006859}}</ref><ref name=":10">{{Cite journal|last=A|first=Yenamandra|date=2016|title=Acute Erythroblastic Leukemia (AEL): A Rare Subset of De Novo AML with A Complex Rearrangement Involving ETV6 Locus and Loss of RB1 Locus|url=https://medcraveonline.com/ICPJL/acute-erythroblastic-leukemia-ael-a-rare-subset-of-de-novo-aml-with-a-complex-rearrangement-involving-etv6-locus-and-loss-of-rb1-locus.html|journal=International Clinical Pathology Journal|volume=2|issue=2|doi=10.15406/icpjl.2016.02.00032}}</ref><ref name=":11">{{Cite journal|last=Fs|first=Khan|date=2017|title=Pure Erythroid Leukemia: The Sole Acute Erythroid Leukemia|url=https://www.heighpubs.org/hbmr/ijbmr-aid1001.php|journal=International Journal of Bone Marrow Research|volume=1|issue=1|pages=001–005|doi=10.29328/journal.ijbmr.1001001}}</ref>. PEL is a distinct entity in the World Health Organization (WHO) classification system within the section of [[Acute Myeloid Leukemia (AML), Not Otherwise Specified]].  PEL is a rare form of acute leukemia with an aggressive clinical course and is characterized by an uncontrolled proliferation of immature erythroid precursors (proerythroblastic or undifferentiated)<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":3" /><ref name=":4" /><ref name=":5" /><ref name=":6" /><ref name=":7" /><ref name=":8" /><ref name=":9" /><ref name=":10" /><ref name=":11" />.

In the 2008 WHO classification, Acute Erythroid leukemia (AEL) was classified into two subtypes: Erythroleukemia (erythroid/myeloid) and Pure Erythroid Leukemia (PEL). However, in the 2016 WHO update, Erythroleukemia was merged into myelodysplastic syndrome, while PEL is now the only type of AEL<ref name=":0">Arber DA, et al., (2008). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, Editors. IARC Press: Lyon, France, p135-136.</ref><ref name=":1">{{Cite journal|last=Qiu|first=Shaowei|last2=Jiang|first2=Erlie|last3=Wei|first3=Hui|last4=Lin|first4=Dong|last5=Zhang|first5=Guangji|last6=Wei|first6=Shuning|last7=Zhou|first7=Chunlin|last8=Liu|first8=Kaiqi|last9=Wang|first9=Ying|date=2017|title=An analysis of 97 previously diagnosed de novo adult acute erythroid leukemia patients following the 2016 revision to World Health Organization classification|url=https://www.ncbi.nlm.nih.gov/pubmed/28793875|journal=BMC cancer|volume=17|issue=1|pages=534|doi=10.1186/s12885-017-3528-6|issn=1471-2407|pmc=5550989|pmid=28793875}}</ref><ref name=":2">{{Cite journal|last=Zuo|first=Zhuang|last2=Polski|first2=Jacek M.|last3=Kasyan|first3=Armen|last4=Medeiros|first4=L. Jeffrey|date=2010|title=Acute erythroid leukemia|url=https://www.ncbi.nlm.nih.gov/pubmed/20807044|journal=Archives of Pathology & Laboratory Medicine|volume=134|issue=9|pages=1261–1270|doi=10.1043/2009-0350-RA.1|issn=1543-2165|pmid=20807044}}</ref><ref name=":3">{{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><ref name=":4">{{Cite journal|last=Zuo|first=Zhuang|last2=Medeiros|first2=L. Jeffrey|last3=Chen|first3=Zhao|last4=Liu|first4=Dingsheng|last5=Bueso-Ramos|first5=Carlos E.|last6=Luthra|first6=Rajyalakshmi|last7=Wang|first7=Sa A.|date=2012|title=Acute myeloid leukemia (AML) with erythroid predominance exhibits clinical and molecular characteristics that differ from other types of AML|url=https://www.ncbi.nlm.nih.gov/pubmed/22844482|journal=PloS One|volume=7|issue=7|pages=e41485|doi=10.1371/journal.pone.0041485|issn=1932-6203|pmc=3402404|pmid=22844482}}</ref><ref name=":5">{{Cite journal|last=Grossmann|first=V.|last2=Bacher|first2=U.|last3=Haferlach|first3=C.|last4=Schnittger|first4=S.|last5=Pötzinger|first5=F.|last6=Weissmann|first6=S.|last7=Roller|first7=A.|last8=Eder|first8=C.|last9=Fasan|first9=A.|date=2013|title=Acute erythroid leukemia (AEL) can be separated into distinct prognostic subsets based on cytogenetic and molecular genetic characteristics|url=https://www.ncbi.nlm.nih.gov/pubmed/23648669|journal=Leukemia|volume=27|issue=9|pages=1940–1943|doi=10.1038/leu.2013.144|issn=1476-5551|pmid=23648669}}</ref><ref name=":6">{{Cite journal|last=Porwit|first=Anna|last2=Vardiman|first2=James W.|date=2011|title=Acute myeloid leukemia with expanded erythropoiesis|url=https://www.ncbi.nlm.nih.gov/pubmed/21880638|journal=Haematologica|volume=96|issue=9|pages=1241–1243|doi=10.3324/haematol.2011.050526|issn=1592-8721|pmc=3166090|pmid=21880638}}</ref><ref name=":7">{{Cite journal|last=Hasserjian|first=Robert P.|last2=Zuo|first2=Zhuang|last3=Garcia|first3=Christine|last4=Tang|first4=Guilin|last5=Kasyan|first5=Armen|last6=Luthra|first6=Rajyalakshmi|last7=Abruzzo|first7=Lynne V.|last8=Kantarjian|first8=Hagop M.|last9=Medeiros|first9=L. Jeffrey|date=2010|title=Acute erythroid leukemia: a reassessment using criteria refined in the 2008 WHO classification|url=https://www.ncbi.nlm.nih.gov/pubmed/20040759|journal=Blood|volume=115|issue=10|pages=1985–1992|doi=10.1182/blood-2009-09-243964|issn=1528-0020|pmc=2942006|pmid=20040759}}</ref><ref name=":8">{{Cite journal|last=Wang|first=Sa A.|last2=Hasserjian|first2=Robert P.|date=2015|title=Acute Erythroleukemias, Acute Megakaryoblastic Leukemias, and Reactive Mimics: A Guide to a Number of Perplexing Entities|url=https://www.ncbi.nlm.nih.gov/pubmed/26071461|journal=American Journal of Clinical Pathology|volume=144|issue=1|pages=44–60|doi=10.1309/AJCPRKYAT6EZQHC7|issn=1943-7722|pmid=26071461}}</ref><ref name=":9">{{Cite journal|last=Wang|first=Wei|last2=Wang|first2=Sa A.|last3=Medeiros|first3=L. Jeffrey|last4=Khoury|first4=Joseph D.|date=2017|title=Pure erythroid leukemia|url=https://www.ncbi.nlm.nih.gov/pubmed/28006859|journal=American Journal of Hematology|volume=92|issue=3|pages=292–296|doi=10.1002/ajh.24626|issn=1096-8652|pmid=28006859}}</ref><ref name=":10">{{Cite journal|last=A|first=Yenamandra|date=2016|title=Acute Erythroblastic Leukemia (AEL): A Rare Subset of De Novo AML with A Complex Rearrangement Involving ETV6 Locus and Loss of RB1 Locus|url=https://medcraveonline.com/ICPJL/acute-erythroblastic-leukemia-ael-a-rare-subset-of-de-novo-aml-with-a-complex-rearrangement-involving-etv6-locus-and-loss-of-rb1-locus.html|journal=International Clinical Pathology Journal|volume=2|issue=2|doi=10.15406/icpjl.2016.02.00032}}</ref><ref name=":11">{{Cite journal|last=Fs|first=Khan|date=2017|title=Pure Erythroid Leukemia: The Sole Acute Erythroid Leukemia|url=https://www.heighpubs.org/hbmr/ijbmr-aid1001.php|journal=International Journal of Bone Marrow Research|volume=1|issue=1|pages=001–005|doi=10.29328/journal.ijbmr.1001001}}</ref>. PEL is a distinct entity in the World Health Organization (WHO) classification system within the section of [[HAEM4:Acute Myeloid Leukemia (AML), Not Otherwise Specified]].  PEL is a rare form of acute leukemia with an aggressive clinical course and is characterized by an uncontrolled proliferation of immature erythroid precursors (proerythroblastic or undifferentiated)<ref name=":0" /><ref name=":1" /><ref name=":2" /><ref name=":3" /><ref name=":4" /><ref name=":5" /><ref name=":6" /><ref name=":7" /><ref name=":8" /><ref name=":9" /><ref name=":10" /><ref name=":11" />.

==Synonyms / Terminology==

==Synonyms / Terminology==