期刊
BLOOD
卷 129, 期 5, 页码 572-581出版社
AMER SOC HEMATOLOGY
DOI: 10.1182/blood-2016-07-726588
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资金
- Cancer Prevention and Research Institute of Texas
- American Lebanese Syrian Associated Charities of St. Jude Children's Research Hospital
- Stand Up to Cancer Innovative Research
- St. Baldrick's Foundation Scholar Award
- American Society of Hematology Scholar Award
- National Institutes of Health, National Cancer Institute [CA21765, HHSN261200800001E, U01 CA157937, CA157937, CA118100]
- Leukemia & Lymphoma Society Specialized Center of Research
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 5.006). 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 5.017), platelet count (HR, 7.437; P 5.005), and Ph-like ALL (HR, 1.818; P 5.03). 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, JAK2mutation wasassociated with inferior outcomes. Our findingsshowhigh frequency of Ph-likeALLin 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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