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Prepublished online as a Blood First Edition Paper on June 12, 2003; DOI 10.1182/blood-2003-02-0434.
Submitted February 10, 2003
Accepted June 5, 2003
AML with 11q23/MLL abnormalities as defined by the WHO-classification: Incidence, partner chromosomes, FAB-subtype, age distribution, and prognostic impact in an unselected series of 1897 cytogenetically analyzed AML
Claudia Schoch*, Susanne Schnittger, Mirjam Klaus, Wolfgang Kern, Wolfgang Hiddemann, and Torsten Haferlach
Laboratory for Leukemia Diagnostics, Department of Internal Medicine III, University Hospital Grosshadern, Ludwig-Maximilians-University, Munich, Germany
* Corresponding author; email: claudia.schoch{at}med3.med.uni-muenchen.de.
AML with 11q23 abnormalities involving the MLL gene comprise one category of recurring genetic abnormalities in the WHO-classification. In an unselected series of 1897 AML cases 54 with an 11q23/MLL rearrangement were identified resulting in an incidence of 2.8%. The incidence of AML with MLL-rearrangement was significantly higher in t-AML than in de novo AML (9.4% vs. 2.6%, p<0.0001). The frequency of MLL-rearrangements was significantly higher in patients younger than 60 years (5.3% vs. 0.8%, p<0.0001). While the incidence of MLL-rearrangements in AML M4, M5a and M5b was 4.7%, 33.3% and 15.9% respectively, it was found in only 0.9% of all other FAB subtypes (p<0.0001). In comparison to AML with intermediate karyotype, AML with 11q23/MLL rearrangement had a worse outcome, which was rather comparable to AML with unfavorable karyotype. Compared to t-AML the median overall survival (OS) of de novo AML with MLL rearrangement was significantly better (2.5 vs. 10 months, p=0.0143). No significant differences in median OS were observed between cases with t(9;11) compared to all other MLL rearrangements (10.0 vs. 8.9 months, p=0.36). In conclusion, the category AML with 11q23/MLL abnormalities accounts for 2.8% of unselected AML, is closely associated with monocytic differentiation and has a dismal prognosis.
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