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Blood, 10 September 2009, Vol. 114, No. 11, pp. 2232-2235.
Prepublished online as a Blood First Edition Paper on June 16, 2009; DOI 10.1182/blood-2009-02-204693.


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CLINICAL TRIALS AND OBSERVATIONS

Brief report

Chronic myeloid leukemia (CML) with P190BCR-ABL: analysis of characteristics, outcomes, and prognostic significance

Dushyant Verma1, Hagop M. Kantarjian1, Dan Jones2, Rajyalakshmi Luthra2, Gautam Borthakur1, Srdan Verstovsek1, Mary Beth Rios1, and Jorge Cortes1

Departments of 1 Leukemia and 2 Hematopathology, The University of Texas M. D. Anderson Cancer Center, Houston

The most common BCR-ABL transcripts in chronic myeloid leukemia (CML) are e13a2(b2a2) and e14a2(b3a2). Other transcripts such as e1a2 are rare and their outcome with tyrosine kinase inhibitors (TKI) therapy is undefined. We analyzed 1292 CML patients and identified 14 with only e1a2 transcripts, 9 in chronic phase (CP), 1 in accelerated phase (AP), and 4 in blast phase (BP). Of the CP, 4 achieved complete hematologic response (CHR); 2, complete cytogenetic response (CCyR); 2, partial cytogenetic response (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 (hyperfractionated cyclophosphamide, vincristine, adriamycin, dexamethasone) plus imatinib/dasatinib or idarubicin plus cytarabine (Ara-C); 2 did not respond, 1 had CCyR, and 1 short-lasting complete molecular response (CMR). Overall, cytogenetic responses lasted 3 to 18 months; only 2 achieved major molecular response (MMR) on TKI. P190BCR-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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Related Article in Blood Online:

Chronic myeloid leukemia with p190BCR-ABL: prevalence, morphology, tyrosine kinase inhibitor response, and kinase domain mutation analysis
Animesh Pardanani, Ayalew Tefferi, Mark R. Litzow, Clive Zent, William J. Hogan, Rebecca F. McClure, and David Viswanatha
Blood 2009 114: 3502-3503. [Full Text] [PDF]



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A. Pardanani, A. Tefferi, M. R. Litzow, C. Zent, W. J. Hogan, R. F. McClure, and D. Viswanatha
Chronic myeloid leukemia with p190BCR-ABL: prevalence, morphology, tyrosine kinase inhibitor response, and kinase domain mutation analysis
Blood, October 15, 2009; 114(16): 3502 - 3503.
[Full Text] [PDF]



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