acute leukemia of ambiguous lineage

Mixed-Phenotype Acute Leukemia: Clinical and Laboratory Features, and Prognosis

MYELOID TUMORS , , ,

AS Antipova1, OYu Baranova1, MA Frenkel’1, NN Tupitsyn1, NA Kupryshina1, TN Obukhova2, AD Shirin1

1 N.N. Blokhin Russian Cancer Research Center, 24 Kashirskoye sh., Moscow, Russian Federation, 115478

2 Hematology Research Center, 4а Novyi Zykovskii pr-d, Moscow, Russian Federation, 125167

For correspondence: Ol’ga Yur’evna Baranova, 24 Kashirskoye sh., Moscow, Russian Federation, 115478; Tel.: +7(499)324-28-64; e-mail: baranova-crc@mail.ru

For citation: Antipova AS, Baranova OYu, Frenkel’ MA, et al. Mixed-Phenotype Acute Leukemia: Clinical and Laboratory Features, and Prognosis. Clinical oncohematology. 2015;8(2):136–50 (In Russ).

ABSTRACT

Objective. To determine clinical and laboratory features and prognosis of mixed-phenotype acute leukemia (MPAL).

Methods. Of 208 AL patients treated in the N.N. Blokhin Russian Cancer Research Center over past 14 years, MPAL was diagnosed in 5 cases (2.4 %). In total 13 patients were enrolled in this study; these patients were hospitalized in the N.N. Blokhin Russian Cancer Research Center (n = 5) and in four other hematological hospitals of Moscow (n = 8). The disease was diagnosed in accordance with the 2008 WHO classification. The median age was 48 years (ranged from 20 to 75 years).

Results. В/М-phenotype was diagnosed in most patients (n = 11) and Т/М only in 2 patients. Translocation t(9;22)(q34;q11) was the most common chromosome aberration diagnosed in 5 (55.5 %) patients. BCR-ABL chimeric gene was in 8 of 9 patients. Treatment strategy was determined by molecular biological and cytogenetic MPAL profiles. Patients with t(9;22)(q34;q11) and/or BCR-ABL chimeric gene treated with imatinib combined with ALL-regimes (n = 8). Patients with Ph-negative MPAL (n = 1) or unknown molecular biological and cytogenetic MPAL profiles (n = 4) received AML-directed therapy or combined regimes for the treatment of ALL and AML. Complete remission (CR) was obtained in most patients (83.3 %) with low rate of early mortality (8.3 %). 3-year OS was 18.2 % (median 14 months), 3-year RFS was 12.8 % (median 16 months). CRs were induced in all Ph+ MPAL patients.

Conclusion. There are no specific clinical and laboratory predictors of MPAL. Tyrosine kinase inhibitors (TKI) play the key role in the treatment of Ph+ MPAL. TKI combined with low intensity ALL regimes seem more promising. The problem of treatment of Ph-negative MPAL patients remains unsolved.

Keywords: mixed-phenotype acute leukemia, acute leukemia of ambiguous lineage, bilineage leukemia, biphenotypic leukemia.

Received: January 10, 2015

Accepted: January 29, 2015

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