Journal of Clinical Oncology, Vol 15, 1803-1810, Copyright © 1997 by American Society of Clinical Oncology
Second cancer risk in hairy cell leukemia: analysis of 350 patients
R Kurzrock, SS Strom, E Estey, S O'Brien, MJ Keating, H Jiang, T Adams and M Talpaz
Department of Bioimmunotherapy, University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
PURPOSE: The discovery of effective therapy for hairy cell leukemia (HCL)
has increased the relevance of long-term outcome. We have therefore
examined the incidence of second cancers. PATIENTS AND METHODS: Data on 350
HCL patients was obtained from the M.D. Anderson Cancer Center Cancer
Registry's computerized data base and from chart review. Standardized
incidence ratios (SIRs) and standardized mortality ratios (SMRs)
(observed/expected [O/E]) were calculated with the expected number
determined using age, sex, and calendar-year-specific rates from the
Connecticut Tumor Registry and from national mortality data, respectively.
RESULTS: The median age of the patients was 50 years and the median
follow-up duration was 6 years. Twenty-six patients developed a second
cancer at least 6 months after the HCL diagnosis (O/E ratio, 1.34; P =
.08). There was no excess of malignancy among patients treated with
interferon alfa (IFN-alpha; P = .27), 2- chlorodeoxyadenosine (2CDA; P =
.37), or deoxycoformycin (DCF; P = .7). However, an excess of
myeloma-related neoplasms (O/E, 13.04; P < .001) and lymphomas (O/E,
8.7; P = .03) was observed. Survival from the advent of systemic therapy
for HCL was better than before this time (P = .0009). Nevertheless,
mortality remained excessive (O/E, 6.17; P < .001), mainly because of
HCL-related infections and secondary malignancy. CONCLUSION: Among 350
patients with HCL, there was an increase in the number of second cancers;
however, it did not reach statistical significance and was not associated
with therapy. The incidence of lymphoid neoplasms was significantly higher
than expected. Survival since the advent of effective systemic therapy was
excellent, although excess mortality was still observed.
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