L. Pagano et al., Clinical and biological features of acute myeloid leukaemia occurring as second malignancy: GIMEMA archive of adult acute leukaemia, BR J HAEM, 112(1), 2001, pp. 109-117
Between July 1992 and June 1996, 3934 new cases of acute leukaemia were reg
istered in the Gruppo Italiano Malattie Ematologiche Maligne dell'Adulto (G
IMEMA) Archive of Adult Acute Leukaemia. Two hundred cases (5.1%) presented
with a history of primary malignancy (PM), 179 of which were acute myeloid
leukaemia (AML). The median age of these cases was significantly higher th
an that of other primitive AML (63 years vs. 57 years; P < 0.001). The numb
er of men was significantly lower than the number of women [74/1544 (4.8%)
vs. 105/1420 (7.4%); odds ratio (OR) 0.63, 95% confidence interval (CI) 0.4
6-0.87; P < 0.002], as was the number of patients aged < 65 years [104/1963
(5.3%) vs. 75/1001 (7.5%); OR 0.69, 95% CI 0.50-0.95; P < 0.01]. An increa
sed incidence of cancer was observed among first-degree relatives of patien
ts with AML occurring after a PM (secondary AML; sAML) [66/179 (36.9%) sAML
vs. 757/2785 (27.2%) de novo AML, age adjusted; OR 2.62, 95% CI 1.07-6.42;
P < 0.005]. Prevalent types of PM were breast cancer, lymphoma and Hodgkin
's disease. sAML occurred after a median latency of 52 months (range 2-379)
. Of the 122 patients who received chemotherapy for sAML, 67 patients (55%)
achieved a complete remission (CR), three a partial remission, 15 (12%) di
ed in induction and 37 (30%) were unresponsive. The median duration of CR w
as 30 weeks (range 4-250). The median overall survival was 7 months (range
1-196). Comparing acute promyelocytic leukaemia with all other French-Ameri
can-British (FAB) groups, a significant increase in CR achievement was obse
rved [14/18 (77.7%) vs. 53/101 (52.4%), P < 0.046] as well as in median CR
duration (55 vs. 24 months, P < 0.02). The analysis of our data suggests th
at not only previous chemotherapy but also genetic predisposition could pla
y a role in the pathogenesis of sAML.