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The category of "refractory anemia with excess blasts in transformation" (RAEB-T) has been abandoned in the new WHO-classification of myelodysplastic syndromes (MDS). The majority of patients previously belonging to this category are now classified as acute myeloid leukaemia (AML). In the FAB-classification, patients had been assigned to the RAEB-T category if they had either (1) a medullary blast count between 20 and 30% or (2) a peripheral blast count of at least 5%, or (3) Auer rods detectable, irrespective of the blast count. We analyzed these subtypes of RAEB-T in terms of hematological characteristics, karyotype anomalies, and prognosis. Patients with more than 20% medullary blasts and patients with at least 5% peripheral blasts as the sole defining parameter for RAEB-T had a median survival of 6 months, as compared to 11 months in patients with Auer rods as the sole defining parameter. The presence of Auer rods therefore does not convey a particularly bad prognosis and does not justify placing patients in a high-risk category of MDS or even classifying them as AML. This finding supports the elimination of Auer rods as a parameter for classification in the new WHO system. On the other hand, the reclassification into RAEB II (according to WHO proposals) of previous RAEB-T patients with a peripheral blast count of at least 5% is problematic, because this feature predicts a median survival not different from that of AML patients.

Citation

Corinna Strupp, Norbert Gattermann, Aristoteles Giagounidis, Carlo Aul, Barbara Hildebrandt, Rainer Haas, Ulrich Germing. Refractory anemia with excess of blasts in transformation: analysis of reclassification according to the WHO proposals. Leukemia research. 2003 May;27(5):397-404

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PMID: 12620291

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