BACKGROUND. There are few data describing the epidemiologic aspects of
Waldenstrom's macroglobulinemia (WM), a rare lymphoplasmaproliferativ
e disorder. METHODS. The authors evaluated the incidence of WM reporte
d in 11 population-based cancer registries in the U.S. RESULTS. A tota
l of 624 cases were diagnosed between January 1, 1988 (when WM became
reportable) and December 31, 1994. Age-adjusted incidence rates for WM
(per 1 million person-years at risk) were 3.4 among males and 1.7 amo
ng females. The rates increased sharply with age, from 0.1 at age < 45
years to 36.3 at age 75+ years (males) and from 0.1 at age < 45 years
to 16.4 at age 75+ years (females). The rates for WM were comparable
to those for hairy cell leukemia, but considerably lower than those fo
r multiple myeloma or chronic lymphocytic leukemia. Some geographic va
riation was evident, with age-adjusted rates among white males ranging
from 2.2-7.8 across registries. There was no significant change in ra
tes over the 7-year study period (P > 0.05). The markedly higher rates
for WM among whites than blacks stand in contrast to multiple myeloma
, which occurs twice as often among blacks. CONCLUSIONS. This survey p
rovides new data regarding the incidence patterns of WM in the U.S. Ho
wever, further epidemiologic studies with biomarkers are needed to def
ine the environmental, genetic, immunologic, and viral determinants of
this rare but distinctive disorder. (C) 1998 American Cancer Society.