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. 2017 Nov;179(4):618-626.
doi: 10.1111/bjh.14953. Epub 2017 Oct 18.

The presence of Philadelphia chromosome does not confer poor prognosis in adult pre-B acute lymphoblastic leukaemia in the tyrosine kinase inhibitor era - a surveillance, epidemiology, and end results database analysis

Igwe J Igwe  1 , Dongyun Yang  2 , Akil Merchant  1 , Noah Merin  3 , George Yaghmour  1 , Kevin Kelly  1 , Giridharan Ramsingh  1
Affiliations

The presence of Philadelphia chromosome does not confer poor prognosis in adult pre-B acute lymphoblastic leukaemia in the tyrosine kinase inhibitor era - a surveillance, epidemiology, and end results database analysis

Igwe J Igwe et al. Br J Haematol. 2017 Nov.

Abstract

The BCR-ABL1 fusion gene is caused by a translocation between chromosomes 9 and 22, resulting in an abnormal chromosome 22 (Philadelphia chromosome; Ph). Prior to the introduction of tyrosine kinase inhibitors (TKI), the presence of BCR-ABL1 conferred a poor prognosis in patients with acute lymphoblastic leukaemia (ALL). We compared the survival of Ph+ and Ph-ALL during the period when TKIs were universally available in the US for Ph+ALL, using a Surveillance, Epidemiology, and End Results (SEER) Database analysis. A total of 2694 patients with pre-B ALL (206 Ph+ALL; 2488 Ph-ALL) aged ≥18 years, who were diagnosed between 2010 and 2014, were identified in SEER registries. The median overall survival (OS) was 32 months in Ph+ALL (95% confidence interval [CI] 18 months-not reached) and 27 months (95% CI 24-30 months) in Ph-ALL (Log-rank test P-value 0·34). Older age was associated with worse prognosis in both Ph+ALL and Ph-ALL. Age-adjusted OS was inferior in Hispanics and African-Americans compared to non-Hispanic whites. Survival of pre-B ALL shows continued improvement with time. Philadelphia chromosome status does not confer poor prognosis in pre-B ALL in the TKI era: prognostic factors in pre-B ALL should be re-evaluated in the light of this finding.

Keywords: SEER; acute lymphoblastic leukaemia; philadelphia chromosome; survival; tyrosine kinase inhibitors.

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Figures

Fig 1.
Fig 1.
Patient selection diagram for pre-B ALL patients from SEER 1973 to 2014. ALL, acute lymphoblastic leukaemia; SEER, surveillance, epidemiology, and end results database.
Fig 2.
Fig 2.
Improvement in 5-year overall survival from 1973 to 2014. Adjusted overall curves of pre-B acute lymphoblastic leukaemia (ALL) from 1973 to 2014. Estimated rates of overall survival for 5 years are illustrated according to the decade of diagnosis.
Fig 3.
Fig 3.
No significant difference in survival between Ph+ALL and Ph−ALL. (A) Kaplan–Meier curves of overall survival (OS) for Ph+ALL compared to Ph−ALL from 2010 to 2014. Estimated 5-year OS rates are illustrated comparing Ph+ALL and Ph−ALL. (B) Estimated 5-year OS of Ph+ALL compared to Ph−ALL diagnosed in 2010–2012. (C) Estimated 2-year survival of Ph+ALL compared to Ph−ALL diagnosed in 2013–2014.
Fig 4.
Fig 4.
Kaplan–Meier curves of overall survival of Ph+ALL compared to Ph−ALL in age group 18–39 years (A), and in patients aged over 40 years (B).
See this image and copyright information in PMC

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