Analysis of T-Cell Receptor V-Beta 2 in Peripheral Blood Lymphocytes as a Diagnostic Marker for Kawasaki Disease

Abstract.

Background: Kawasaki disease (KD) is the most frequent cause of acquired heart disease in children, yet there are no specific diagnostic markers for it. There is controversy whether and by what mechanism selective expansion of T-cell subsets occurs and whether this phenomenon might be helpful for early diagnosis.

Patients and Methods: To obtain age-related normal values of V-beta 2⁺ T-lymphocytes in healthy children and to investigate expansion in KD, we measured expression in 228 children. Peripheral CD3⁺ cells were stained with monoclonal antibodies to V-beta 2.1 and the percentage of V-beta 2⁺ T-cells was analyzed using flow cytometry.

Results: In a control group of 184 healthy subjects (0 to 17 years; median age 6.0 years) we found age-related changes. Levels were highest from 0–2 years (9.02±2.80%) and declined towards adolescence (7.56±2.42% in 11–15 year olds) (p < 0.001). Results were compared with 24 patients with acute febrile diseases of origin other than KD and with 20 patients with presumed diagnosis KD at admission. Interestingly, while all seven patients whose clinical picture retrospectively confirmed KD showed elevated levels, none of the other children, including none of the patients with initially suspected but not clinically confirmed KD, had levels higher than normal, with the exception of two teenage girls with toxic shock syndrome, a classical superantigen-mediated disease.

Conclusion: When compared with appropriate age-matched controls, estimation of V-beta 2⁺ T-cells might be a valuable tool when making diagnostic decisions suspecting KD.