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Multicenter Study
. 2010 Mar 11;115(10):1985-92.
doi: 10.1182/blood-2009-09-243964. Epub 2009 Dec 29.

Acute erythroid leukemia: a reassessment using criteria refined in the 2008 WHO classification

Robert P Hasserjian  1 , Zhuang Zuo, Christine Garcia, Guilin Tang, Armen Kasyan, Rajyalakshmi Luthra, Lynne V Abruzzo, Hagop M Kantarjian, L Jeffrey Medeiros, Sa A Wang
Affiliations
Multicenter Study

Acute erythroid leukemia: a reassessment using criteria refined in the 2008 WHO classification

Robert P Hasserjian et al. Blood. .

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.

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Figures

Figure 1
Figure 1
Overall survival (OS) of 106 acute erythroid leukemic patients by treatment modalities. The 21 patients who underwent allogeneic bone marrow transplantation (BMT) had a superior OS (median, 23 months) compared with 52 patients who received induction chemotherapy without BMT (median, 9 months; P = .015) as well as 33 patients who did not receive high-intensity chemotherapy (median OS, 4 months; P = .002). There was no difference in OS between patients receiving induction versus those who did not receive high-intensity chemotherapy (P = .10).
Figure 2
Figure 2
Overall survival (OS) of acute erythroid leukemia (AEL) patients by clinical subgroups. The therapy-related AEL patients (T-AEL) had a median OS of 4.5 months, inferior to that of de novo AEL patients (N-AEL; median OS, 11 months; P < .001) and patients with AEL after an antecedent myelodysplastic syndrome or chronic cytopenia (MDS-AEL; median OS, 17 months; P = .001). There was no difference in OS between the N-AEL and MDS-AEL patients (P = .99).
Figure 3
Figure 3
Overall survival (OS) of acute erythroid leukemia (AEL) patients by cytogenetic risk grouping. (A) The median OS of patients in the UKMRC AML intermediate and unfavorable groups was 30 and 6 months, respectively (P < .001). (B) The median OS of patients in the IPSS MDS good-, intermediate-, and poor-risk groups was 30, 23, and 6 months, respectively. The poor IPSS MDS risk group had a significantly inferior OS compared with the intermediate and good groups (both P < .001). There was no significant difference in OS between patients in the IPSS MDS good- and intermediate-risk groups (P = .68).
Figure 4
Figure 4
Comparison of 40 patients who had MDS with erythroid hyperplasia (MDS-E), 41 patients who had AML with myelodysplasia-related changes and erythroid hyperplasia (AML-MRC), and 124 patients with acute erythroid leukemia (AEL). The median OS values are 14, 8, and 10 months, respectively, which are not significantly different (P = .313).
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