Acute Erythroid Leukemia

Primary Author(s)*

Ashwini Yenamandra PhD FACMG

Cancer Category/Type

Acute Myeloid Leukemia

Cancer Sub-Classification / Subtype

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 [1-12].

Definition / Description of Disease

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) [1-12]. Erythroleukemia (erythroid/myeloid) may be de novo or evolved from myeloid or sometimes from myeloproliferative neoplasms (MPN) [1,10].



1. Arber DA, et al., (2008). Acute myeloid leukaemia with recurrent genetic abnormalities, in 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.

2. Shaowei Qiu, et al., (2017). An analysis of 97 previously diagnosed de novo adult acute erythroid leukemia patients following the 2016 revision to World Health Organization classification. BMC Cancer 17: 544, PMID 28793875.

3. Zhuang Zuo, et al., (2010). Acute Erythroid Leukemia (Review Article). Arch Pathol Lab Med 134:1261-1270, PMID 20807044.

4. Arber DA, et al., (2016). The updated WHO classification of Hematological malignancies. The 2016 revision to the World Health organization classification of myeloid neoplasms and acute leukemia (Review Series). Blood 127(20):2391-2405, PMID 27069254.

5. Zuo Z, et al., (2012). Acute myeloid leukemia (AML) with erythroid predominance exhibits clinical and molecular characteristics that differ from other types of AML. PLoS One 7(7):e41485, PMID 22844482.

6. Grossmann V, et al., (2013). Acute erythroid leukemia (AEL) can be separated into distinct prognostic subsets based on cytogenetic and molecular genetic characteristics. Leukemia 27(9): 1940-1943, PMID 23648669.

7. Porwit A, et al., (2011). Acute myeloid leukemia with expanded erythropoiesis. Haematologica 96(9):1241-1243, PMID 21880638.

8. Hasserjian RP, et al., (2010). Acute erythroid leukemia: a reassessment using criteria refined in the 2008 WHO classification. Blood 115(10):1985-1992, PMID 20040759.

9. Wang SA, et al., (2015). Acute erythroleukemias, acute megakaryoblastic leukemias, and reactive mimics: a guide to a number of perplexing entities. Am J Clin Pathol 144(1):44-60, PMID 26071461.

10. Wang W, et al., (2016). Acute Erythroid Leukemia. Am J Hematol. 2017; 92:292–296, PMID 2800685.

11. Yenamandra A, et al., (2016). Acute Erythroblastic Leukemia (AEL): A rare subset of de novo AML with a complex rearrangement involving ETV6 locus and loss of RB1 locus. Int Clin Pathol J 2(2): 00032, DOI: 10.15406/icpjl.2016.02.00032.

12. Fauzia SF, et al., (2017). Pure erythroid leukemia: The sole acute erythroid leukemia. Int J Bone Marrow Res 1: 001-05 (open access).


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