Abstract
We have reported that IgA plasma cells infiltrate vascular and some nonvascular tissues in fatal acute Kawasaki Disease (KD). The presence of these cells in vascular tissue might reflect a large increase in circulating IgA B lymphocytes in acute KD, with non-specific entry of these cells into vascular tissue along with other inflammatory cells in the peripheral blood. To determine whether peripheral blood IgA B lymphocytes are increased in acute KD, we performed three-color flow cytometry to detect surface and cytoplasmic immunoglobulin expression (IgA, IgM, IgD, and IgG) of peripheral B lymphocytes in 15 KD patients during the acute, subacute, and convalescent stages of illness and in age-matched febrile (n=10) and afebrile (n=15) controls. Unexpectedly, absolute numbers of peripheral blood B lymphocytes expressing surface IgA were significantly lower in acute KD compared with febrile (p=0.01) and afebrile (p<0.001) age-matched controls. Absolute numbers of B lymphocytes expressing cytoplasmic IgA were also lower in acute KD compared with febrile (p=0.003) and afebrile (p<0.001) age-matched controls. Additionally, absolute numbers of B lymphocytes expressing cytoplasmic IgG was significantly lower in acute KD patients compared with febrile (p=0.02) and afebrile (p<0.001) age-matched controls. There were no differences among groups in absolute numbers of B lymphocytes expressing surface IgG, surface and cytoplasmic IgM, and surface and cytoplasmic IgD. These findings indicate that IgA plasma cells are not present in KD tissue as a result of excess numbers of IgA B lymphocytes in peripheral blood. We speculate that IgA B lymphocytes selectively move from the peripheral circulation into KD target tissues as a part of a specific IgA immune response.
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Shingadia, D., O'Gorman, M., Rowley, A. et al. Surface and Cytoplasmic Immunoglobulin Expression in Circulating B-lymphocytes in Acute Kawasaki Disease. Pediatr Res 53, 173 (2003). https://doi.org/10.1203/00006450-200301000-00115
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DOI: https://doi.org/10.1203/00006450-200301000-00115