Transient response to imatinib mesylate (STI571) in a patient with the ETV6-ABL t(9;12) translocation.

Abstract:

:We report the transient response of a patient with the ETV6-ABL fusion gene to imatinib mesylate (STI571). A 38-year-old man was referred with an erroneous diagnosis of Philadelphia-positive chronic myeloid leukemia in blastic transformation for treatment with the ABL tyrosine kinase inhibitor, STI571. Further investigation indicated that the patient in fact had acute myeloid leukemia; no evidence of the Philadelphia translocation or BCR-ABL was found using fluorescence in situ hybridization (FISH) or reverse transcription-polymerase chain reaction. Detailed FISH analysis identified a cryptic t(9;12) translocation, and molecular studies confirmed the presence of the ETV6-ABL fusion transcript. Because the patient was gravely ill at presentation, treatment was commenced immediately with STI571 monotherapy, resulting in considerable initial improvement. However within 10 days the patient's condition again deteriorated, and he required conventional chemotherapy. This case has implications for the design of future studies using STI571 in leukemias involving ABL-encoded fusion proteins other than BCR-ABL.

journal_name

Blood

journal_title

Blood

authors

O'Brien SG,Vieira SA,Connors S,Bown N,Chang J,Capdeville R,Melo JV

doi

10.1182/blood.v99.9.3465

subject

Has Abstract

pub_date

2002-05-01 00:00:00

pages

3465-7

issue

9

eissn

0006-4971

issn

1528-0020

journal_volume

99

pub_type

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