Table 5.

Prevalence of severe infections, gingivitis, and leukemic transformation by ELA2 genotype



SCN group

CN group

NN
NM
P
NN
NM
P
No. patients   35   19   —   15   12   —  
Period of follow-up without G-CSF*  9.4   4.1   —   7.4   8.2   —  
Prevalence of infections, n       
   All infections (%)   15 (42)   16 (84)   .003   3 (20)   9 (75)   .006  
   Omphalitis   3   4   NS   0   1   NS  
   Cellulitis   7   11   .006   1   4   NS  
   Perianal abscess   5   5   NS   0   2   NS  
   Pneumonia   6   11   .003   2   2   NS  
   Sepsis   1   3   NS   1   2   NS  
   Aspergillosis   3   2   NS   3   1   NS  
Prevalence of gingivitis, n (%)   17 (49)   11 (58)   NS   5 (33)   5 (42)   NS  
Prevalence of leukemic transformation, n (%)
 
0
 
4 (21)
 
.04
 
0
 
0
 

 


SCN group

CN group

NN
NM
P
NN
NM
P
No. patients   35   19   —   15   12   —  
Period of follow-up without G-CSF*  9.4   4.1   —   7.4   8.2   —  
Prevalence of infections, n       
   All infections (%)   15 (42)   16 (84)   .003   3 (20)   9 (75)   .006  
   Omphalitis   3   4   NS   0   1   NS  
   Cellulitis   7   11   .006   1   4   NS  
   Perianal abscess   5   5   NS   0   2   NS  
   Pneumonia   6   11   .003   2   2   NS  
   Sepsis   1   3   NS   1   2   NS  
   Aspergillosis   3   2   NS   3   1   NS  
Prevalence of gingivitis, n (%)   17 (49)   11 (58)   NS   5 (33)   5 (42)   NS  
Prevalence of leukemic transformation, n (%)
 
0
 
4 (21)
 
.04
 
0
 
0
 

 

NS indicates not significant; NN genotype, patient with no ELA2 mutation; NM genotype, patient with a heterozygous ELA2 mutation; and —, not applicable.

*

Expressed as the median time in years