| Citace |
KAYSER, Sabine, Robert K. HILLS, Marlise R. LUSKIN, Andrew M.
BRUNNER, Christine TERRE, Jorg WESTERMANN, Kamal MENGHRAJANI,
Carole SHAW, Maria R. BAER, Michelle A. ELLIOTT, Alexander E.
PERL, Zdeněk RÁČIL, Jiří MAYER, Pavel ZAK, Tomas SZOTKOWSKI,
Stephane DE BOTTON, David GRIMWADE, Karin MAYER, Roland B.
WALTER, Alwin KRAMER, Alan K. BURNETT, Anthony D. HO, Uwe
PLATZBECKER, Christian THIEDE, Gerhard EHNINGER, Richard M.
STONE, Christoph ROLLIG, Martin S. TALLMAN, Elihu H. ESTEY,
Carsten MULLER-TIDOW, Nigel H. RUSSELL, Richard F. SCHLENK a
Mark J. LEVIS. Allogeneic hematopoietic cell transplantation
improves outcome of adults with t(6;9) acute myeloid leukemia:
results from an international collaborative study.
Haematologica. PAVIA: FERRATA STORTI FOUNDATION, 2020, roč.
105, č. 1, s. 161-169. ISSN 0390-6078. Dostupné z:
https://dx.doi.org/10.3324/haematol.2018.208678. |
| Popis |
A cute myeloid leukemia (AML) with t(6;9)(p22;q34) is a distinct entity accounting for 1-2% of AML cases. A substantial proportion of these patients have a concomitant FLT3-ITD. While outcomes are dismal intensive chemotherapy, limited evidence suggests allogeneic hematopoietic cell transplantation (allo-HCT) may improve survival if performed early during first complete remission. We report on a cohort of 178 patients with t(6;9)(p22;q34) within an international, multicenter collaboration. Median age was 46 years (range: 16-76), AML was de novo in 88%, FLT3-ITD was present in 62%, and additional cytogenetic abnormalities in 21%. Complete remission was achieved in 81% (n=144), including 14 patients who received high-dose cytarabine after initial induction failure. With a median follow up of 5.43 years, estimated overall survival at five years was 38% (95%CI: 31-47%). Allo-HCT was performed in 117 (66%) patients, including 89 in first complete remission. Allo-HCT in first complete remission was associated with higher 5-year relapse-free and overall survival as compared to consolidation chemotherapy: 45% (95%CI: 35-59%) and 53% (95%CI: 42-66%) versus 7% (95%CI: 3-19%) and 23% (95%CI: 13-38%), respectively. For patients undergoing allo-HCT, there was no difference in overall survival rates at five years according to whether it was performed in first [53% (95%CI: 42-66%)], or second [58% (95%CI: 31-100%); n=10] complete remission or with active disease/relapse [54% (95%CI: 34-84%); n=18] (P=0.67). Neither FLT3-ITD nor additional chromosomal abnormalities impacted survival. In conclusion, outcomes of t(6;9)(p22;q34) AML are poor with chemotherapy, and can be substantially improved with allo-HCT.
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