HLA-A*31:01 was reported to be associated with carbamazepine (CBZ)-induced 
severe cutaneous adverse reactions (SCAR), including drug reaction with 
eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome (SJS) and 
toxic epidermal necrolysis (TEN). We conducted an international study using 
consensus diagnosis criteria to enroll a total of 93 patients with CBZ-SCAR from 
Europe or Asia. We found that HLA-A*31:01 showed a significant association with 
CBZ-DRESS in Europeans (P<0.001; odds ratio (OR) (95% confidence interval 
(CI))=57.6 (11.0-340)), and the strong association was also found in Chinese 
(P<0.001; OR (95% CI)=23.0 (4.2-125)). However, HLA-A*31:01 had no association 
with CBZ-SJS/TEN in neither Chinese nor Europeans. By comparison, HLA-B*15:02 
showed a strong association with CBZ-SJS/TEN in Chinese (P<0.001, OR (95% 
CI)=58.1 (17.6-192)). A meta-analysis of this and other published studies 
confirmed that in all populations, HLA-A*31:01 had an extremely strong 
association with CBZ-DRESS (P<0.001, a pooled OR (95% CI)=13.2 (8.4-20.8)), but 
a much weaker association with CBZ-SJS/TEN (P=0.01, OR (95% CI)=3.94 
(1.4-11.5)). Our data revealed that HLA-A*31:01 is a specific predictor for 
CBZ-DRESS but not for CBZ-SJS/TEN. More studies are needed to investigate the 
genetic determinant of CBZ-SJS/TEN in Europeans. Considering the potential 
clinical utility, the cost-effectiveness of the combined HLA-A*31:01 and 
HLA-B*15:02 genetic test to prevent CBZ-SCAR in Chinese needs further 
investigation.