Refractory anemia with excess of blasts in transformation: analysis of reclassification according to the WHO proposals.
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.
Strupp
Corinna
C
Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Moorenstreet 5, 40225 Düsseldorf, Germany. c.strupp@web.de
Gattermann
Norbert
N
Giagounidis
Aristoteles
A
Aul
Carlo
C
Hildebrandt
Barbara
B
Haas
Rainer
R
Germing
Ulrich
U
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000754:Anemia, Refractory, with Excess of Blasts / Q000097:blood / Q000145:classification* / Q000401:mortality / Q000473:pathology; D001772:Blood Cell Count; D002471:Cell Transformation, Neoplastic; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D007621:Karyotyping; D007770:L-Lactate Dehydrogenase / Q000097:blood; D007951:Leukemia, Myeloid / Q000097:blood / Q000145:classification* / Q000401:mortality / Q000473:pathology; D015993:Life Tables; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000145:classification*; D009363:Neoplasm Proteins / Q000097:blood; D014411:Neoplastic Stem Cells / Q000473:pathology; D011379:Prognosis; D012306:Risk; D016019:Survival Analysis; D014944:World Health Organization
D009363:Neoplasm Proteins; D007770:L-Lactate Dehydrogenase