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. 2017 Sep 8;7(9):e605.
doi: 10.1038/bcj.2017.81.

Secondary acute lymphoblastic leukemia is a distinct clinical entity with prognostic significance

A S Rosenberg  1   2 , A Brunson  1 , J K Paulus  3 , J Tuscano  2   4 , T Wun  1   2   4 , T H M Keegan  1   2 , B A Jonas  1   2   4
Affiliations

Secondary acute lymphoblastic leukemia is a distinct clinical entity with prognostic significance

A S Rosenberg et al. Blood Cancer J. .

Abstract

The effect of prior malignancy on the risk of developing, and prognosis of, acute lymphoblastic leukemia (ALL) is unknown. This observational study utilized the California Cancer Registry to estimate the risk of developing ALL after a prior malignancy using standardized incidence ratios (SIRs, 95% confidence intervals). ALL occurring after a malignancy with an SIR>1 (increased-risk (IR) malignancies) was considered secondary ALL (s-ALL). Adjusted hazard ratios (aHRs, 95% confidence intervals) compared the effect of s-ALL with de novo ALL on overall survival. A total of 14 481 patients with ALL were identified (1988-2012) and 382 (3%) had a known prior malignancy. Any prior malignancy predisposed patients to developing ALL: SIR 1.62 (1.45-1.79). Hematologic malignancies (SIR 5.57, 4.38-6.98) and IR-solid tumors (SIR 2.11, 1.73-2.54) increased the risk of developing ALL. s-ALL increased the risk of death compared with de novo ALL (aHR 1.38 (1.16-1.63)) and this effect was more pronounced among younger patients (age<40 years: aHR 4.80 (3.15-7.30); age⩾40 years: aHR 1.40 (1.16-1.69)) (interaction P<0.001). This population-based study demonstrates that s-ALL is a distinct entity that occurs after specific malignancies and carries a poor prognosis compared with de novo ALL, particularly among patients <40 years of age.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
SIRs for acute lymphoblastic leukemia preceded by prior malignancy. IR solid tumors defined as those solid tumors with SIRs >1. Older adults are defined as age ⩾40 years and pediatric/AYA are defined as age <40 years. Lower 95% confidence intervals are noted by the whisker.
Figure 2
Figure 2
SIRs for acute lymphoblastic leukemia preceded by prior chemotherapy/radiation therapy. IR solid tumors defined as those solid tumors with SIRs >1. Prior treatment refers treatment with chemotherapy and/or radiation therapy for the prior malignancy. Older adults are defined as age ⩾40 years and pediatric/AYA are defined as age <40 years. Lower 95% confidence intervals are noted by the whisker.
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