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Blood, Vol. 92 No. 4 (August 15), 1998:
pp. 1160-1164
Second Malignancies in Patients With Hairy Cell Leukemia in British
Columbia: A 20-Year Experience
Wing Y. Au,
Richard J. Klasa,
Richard Gallagher,
Nhu Le,
Randy D. Gascoyne, and
Joseph M. Connors
From the Divisions of Medical Oncology, Cancer Control Research, and
Pathology, British Columbia Cancer Agency and the University of British
Columbia, Vancouver, Canada.
The purpose of this study was to compare the relative
risk of second malignancies in a cohort of patients with hairy cell leukemia (HCL) against the normal population. Potential effects of type
of treatment and duration of follow-up and the site distribution of
cancer were also examined. Between 1976 and 1996, 117 patients were
diagnosed with HCL in British Columbia who were referred to the British
Columbia Cancer Agency (BCCA) for treatment. All additional
malignancies were traced using a provincial population-based cancer
registry and follow-up records from the BCCA. There were 90 men and 27 women. Median age at diagnosis was 53 years. The median follow-up time
was 68 months. Twenty-three patients underwent primary splenectomy, 65 received interferon alpha, 24 deoxycoformycin, and 67 cladribine
(2-chlorodeoxyadenosine). Thirty-six patients had an additional
malignancy (30.7%) with a total of 44 tumors. Six patients (5.1%) had
two or more malignancies. Twenty-five patients had malignancies
diagnosed after HCL (21.3%), three concurrent with HCL (2.6%), and 12 preceding HCL (10.2%). Second tumors (n = 28 tumors) occurred at a
median of 40 months after HCL (range, 3 to 167). The relative rate (RR)
of second malignancy among men and women was 2.91 (P < .001)
and 1.65 (P = .23), respectively, compared with age and
secular trend-matched controls. There were eight prostate cancers, nine
nonmelanoma skin cancers, two lung cancers, and four gastrointestinal
adenocarcinomas. The RR (90% confidence interval [CI]) in the
various treatment groups were: splenectomy (RR = 0.21 to 3.81),
purine analogues (RR = 0.60 to 5.69), interferon then purine
analogues (RR = 1.60 to 4.31), interferon alone (RR = 1.57 to
8.40). Cancer risk peaked at 2 years after HCL (RR = 4.13) and fell
steadily afterwards, reaching a RR of 1.82 at 6 years. Twenty patients
died, six due to HCL, 10 due to second malignancies, and four of
unrelated causes. HCL patients appear to be inherently prone to
malignancies. This appears to be more related to HCL tumor burden than
to genetic predisposition or treatment effect. RR tends to fall with
time after effective treatment. However, close monitoring for and
vigorous prevention of cancer in HCL patients is advisable.
© 1998 by The American Society of Hematology.
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