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Blood. 2017 Feb 2;129(5):572-581. doi: 10.1182/blood-2016-07-726588. Epub 2016 Dec 5.

Ph-like acute lymphoblastic leukemia: a high-risk subtype in adults.

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Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX.
Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN.
Department of Hematopathology.
Institute for Personalized Cancer Therapy, and.
Department of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, TX.
Department of Pathology, University of New Mexico Comprehensive Cancer Center, Albuquerque, NM; and.
Division of Hematology/Oncology, Chao Family Comprehensive Cancer Center, University of California Irvine Medical Center, Orange, CA.


Philadelphia chromosome (Ph)-like acute lymphoblastic leukemia (ALL) is a high-risk subtype of ALL in children. There are conflicting data on the incidence and prognosis of Ph-like ALL in adults. Patients with newly diagnosed B-cell ALL (B-ALL) who received frontline chemotherapy at MD Anderson Cancer Center underwent gene expression profiling of leukemic cells. Of 148 patients, 33.1% had Ph-like, 31.1% had Ph+, and 35.8% had other B-ALL subtypes (B-other). Within the Ph-like ALL cohort, 61% had cytokine receptor-like factor 2 (CRLF2) overexpression. Patients with Ph-like ALL had significantly worse overall survival (OS), and event-free survival compared with B-other with a 5-year survival of 23% (vs 59% for B-other, P = .006). Sixty-eight percent of patients with Ph-like ALL were of Hispanic ethnicity. The following were associated with inferior OS on multivariable analysis: age (hazard ratio [HR], 3.299; P < .001), white blood cell count (HR, 1.910; P = .017), platelet count (HR, 7.437; P = .005), and Ph-like ALL (HR, 1.818; P = .03). Next-generation sequencing of the CRLF2+ group identified mutations in the JAK-STAT and Ras pathway in 85% of patients, and 20% had a CRLF2 mutation. Within the CRLF2+ group, JAK2 mutation was associated with inferior outcomes. Our findings show high frequency of Ph-like ALL in adults, an increased frequency of Ph-like ALL in adults of Hispanic ethnicity, significantly inferior outcomes of adult patients with Ph-like ALL, and significantly worse outcomes in the CRLF2+ subset of Ph-like ALL. Novel strategies are needed to improve the outcome of these patients.

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