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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.

Author information

1
Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, TX.
2
Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN.
3
Department of Hematopathology.
4
Institute for Personalized Cancer Therapy, and.
5
Department of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, TX.
6
Department of Pathology, University of New Mexico Comprehensive Cancer Center, Albuquerque, NM; and.
7
Division of Hematology/Oncology, Chao Family Comprehensive Cancer Center, University of California Irvine Medical Center, Orange, CA.

Abstract

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.

PMID:
27919910
PMCID:
PMC5290985
DOI:
10.1182/blood-2016-07-726588
[Indexed for MEDLINE]
Free PMC Article

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