In order to assess the prognostic value of nephromegaly (kidney enlarg
ement greater than or equal to 2 SD from mean for age) in children at
diagnosis of acute lymphoblastic leukemia (ALL), kidney size was inves
tigated by intravenous pyelogram (IP), according to the method of Eklo
f and Ringertz, in 101 children diagnosed with ALL in the period of 19
75-1983. In the same period 103 additional patients with ALL were not
investigated with IP for logistical reasons. Characteristics at the di
agnosis of ALL in the patients of the investigated and non-investigate
d (with IP) groups were quite similar and event-free-survival (EFS) cu
rves superimposable. Eighty-nine (50 males and 39 females, median age
61 months) out of 101 patients underwent IP before starting any therap
y and were evaluated in the study; 27 (30.3%) presented with nephromeg
aly (bilateral in 13 cases and unilateral in 14). With a median follow
-up time of 14.4 years (range 11-19 years), the EFS at 15 years from d
iagnosis was 18.5% (SE 9.5) in the nephromegalic group and 38.4% (SE 6
.4) in the non-nephromegalic group. The association of poorer EFS with
nephromegaly was confirmed when the comparison was adjusted by white
blood cell count and age at diagnosis in a Cox regression model. Nephr
omegaly at diagnosis of childhood ALL may have an independent prognost
ic value in patients treated with 'not intensive' protocols; however,
this finding should be confirmed in patients treated uniformly with co
ntemporary intensive protocols.