Imatinib with intensive chemotherapy in AML with t(9;22)(q34.1;q11.2)/BCR::ABL1. A DATAML registry study
Acute myeloid leukemia (AML) with t(9;22) (q34.1; q11.2)/ BCR::ABL1 , a distinct entity within the group of AML with defining genetic abnormalities, belong to the adverse-risk group of the 2022 ELN classification. However, there is little data on outcome since the era of tyrosine kinase inhibitors....
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Veröffentlicht in: | Blood cancer journal (New York) 2024-05, Vol.14 (1), p.91-10, Article 91 |
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Sprache: | eng |
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Zusammenfassung: | Acute myeloid leukemia (AML) with t(9;22) (q34.1; q11.2)/
BCR::ABL1
, a distinct entity within the group of AML with defining genetic abnormalities, belong to the adverse-risk group of the 2022 ELN classification. However, there is little data on outcome since the era of tyrosine kinase inhibitors. Among 5819 AML cases included in the DATAML registry, 20 patients with de novo
BCR::ABL1
+
AML (0.3%) were identified. Eighteen patients treated with standard induction chemotherapy were analyzed in this study. Imatinib was added to chemotherapy in 16 patients. The female-to-male ratio was 1.25 and median age was 54 years. The t(9;22) translocation was the sole chromosomal abnormality in 12 patients. Main gene mutations detected by NGS were
ASXL1
,
RUNX1
and
NPM1
. Compared with patients with myeloid blast phase of chronic myeloid leukemia (CML-BP), de novo
BCR::ABL1
+
AML had higher WBC, fewer additional chromosomal abnormalities, lower CD36 or CD7 expression and no
ABL1
mutations. Seventeen patients (94.4%) achieved complete remission (CR) or CR with incomplete hematologic recovery. Twelve patients were allografted in first remission. With a median follow-up of 6.3 years, the median OS was not reached and 2-year OS was 77% (95% CI: 50–91). Four out of five patients who were not transplanted did not relapse. Comparison of
BCR::ABL1
+
AML, CML-BP, 2017 ELN intermediate (
n
= 643) and adverse-risk patients (
n
= 863) showed that patients with
BCR::ABL1
+
AML had a significant better outcome than intermediate and adverse-risk patients.
BCR::ABL1
+
AML patients treated with imatinib and intensive chemotherapy should not be included in the adverse-risk group of current AML classifications. |
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ISSN: | 2044-5385 2044-5385 |
DOI: | 10.1038/s41408-024-01069-9 |