Verma D, Kantarjian HM, Jones D, Luthra R, Borthakur G, Verstovsek S, Rios MB, Cortes J
Chronic myeloid leukemia (CML) with P190BCR-ABL - analysis of characteristics, outcomes and prognostic significance. [JOURNAL ARTICLE]
Blood 2009 Jun 16.
The most common BCR-ABL transcripts in CML are e13a2(b2a2) and e14a2(b3a2). Other transcripts like e1a2 are rare and their outcome with TKI therapy is undefined. We analyzed 1292 CML patients and identified 14 with only e1a2 transcripts, 9 in chronic (CP), 1 accelerated (AP), and 4 blast phase (BP). Of the CP, 4 achieved CHR, 2 CCyR, 2 PCyR, and 1 did not respond to imatinib. Five patients progressed to myeloid BP (3), lymphoid BP (1), or AP (1). The AP patient received various TKIs sequentially and achieved only CHR. BP patients received Hyper-CVAD+imatinib/dasatinib or idarubicin+Ara-C; 2 did not respond, 1 had CCyR, and 1 short-lasting CMR. Overall, cytogenetic responses lasted 3-18 months, only 2 achieved MMR on TKI. P190(BCR-ABL) CML is rare and is associated with an inferior outcome to therapy with TKI. These patients need to be identified as high-risk patients.
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