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Acute erythroid leukemia: a reassessment using criteria refined in the 2008 WHO classification
Robert P Hasserjian1, Zhuang Zuo, Christine Garcia
1Department of Pathology, Massachusetts General Hospital, 55 Fruit St, Boston, MA 02114, USA. rhasserjian@partners.org
Abstract:
Acute erythroid leukemia (AEL) is a rare type of acute myeloid leukemia (AML) for which diagnostic criteria have been refined in the 2008 World Health Organization (WHO) classification of AML. The relationship of AEL to myelodysplastic syndromes (MDSs) and to AML with myelodysplasia-related changes (AML-MRC) is not clearly defined. We conducted a retrospective, multi-institutional study of patients with AEL and compared them with patients with MDS or AML-MRC with erythroid hyperplasia (> or = 50% erythroid cells). Among a total of 124 patients with AEL, 32% had a history of MDS or chronic cytopenia, 32% had therapy-related disease, and 35% had de novo disease. Sixty-four percent of patients had unfavorable AML risk-group karyotypes. FLT3 and RAS mutations were infrequent, occurring in 6% and 2%, respectively. The median overall survival (OS) of all AEL patients was 8 months, comparable with that of patients with MDS or AML-MRC with erythroid hyperplasia. The OS was related to cytogenetic risk group, but not blast count or morphologic dysplasia. Our findings suggest that AEL is in the continuum of MDS and AML with erythroid hyperplasia, where karyotype rather than an arbitrary blast cutoff represents the most important prognostic factor.
Insights
Acute erythroid leukemia (AEL) is a rare myeloid leukemia. Karyotype, not blast count, is the key prognostic factor, suggesting AEL is part of a continuum with myelodysplastic syndromes and AML with erythroid hyperplasia.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Acute erythroid leukemia (AEL) is a rare subtype of acute myeloid leukemia (AML).
- Diagnostic criteria for AEL were updated in the 2008 WHO classification.
- The distinction between AEL, myelodysplastic syndromes (MDS), and AML with myelodysplasia-related changes (AML-MRC) requires further clarification.
Purpose of the Study:
- To investigate the relationship between AEL, MDS, and AML-MRC.
- To identify prognostic factors in AEL patients.
Main Methods:
- Retrospective, multi-institutional study.
- Comparison of 124 AEL patients with MDS or AML-MRC patients exhibiting erythroid hyperplasia (≥50% erythroid cells).
Main Results:
- 32% of AEL patients had a history of MDS or chronic cytopenia; 32% had therapy-related disease; 35% had de novo disease.
- 64% of AEL patients had unfavorable AML risk-group karyotypes.
- Median overall survival (OS) was 8 months, similar to MDS/AML-MRC with erythroid hyperplasia.
- OS was significantly related to cytogenetic risk group, but not blast count or morphologic dysplasia.
Conclusions:
- AEL appears to exist on a continuum with MDS and AML with erythroid hyperplasia.
- Cytogenetic risk group is the most critical prognostic factor in AEL, outweighing blast count or morphologic dysplasia.
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