To determine the possible alteration of the inhibitor kappaBalpha (HUGO-approved symbol, NFKBIA) gene in Chinese Hodgkin lymphoma (HL) patients, NFKBIA mRNA and protein expression in 22 primary HL patients were examined. Individual tumor cells were used for amplification to obtain the NFKBIA gene, and the polymerase chain reaction products were sequenced. Compared with reactive surrounding lymphocytes, inhibitor kappaBalpha protein (IkappaBalpha) expression was weaker in the cytoplasm of H-RS (Hodgkin and Reed-Sternberg) cells. NFKBIA mRNA was strongly expressed in H-RS cells from HL sections, and little was detected in the reactive surrounding lymphocytes. A total of 37.5% of the patients with HL had mutations in the NFKBIA gene. Some mutations possibly resulted in C-terminally truncated form of the IkappaBalpha. These data suggest that the impairment of the IkappaBalpha functions was produced during the pathogenesis of the tumor cell clone in Chinese HL patients.