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  • Title: Kawasaki disease associated with reactive hemophagocytic lymphohistiocytosis.
    Author: Cummings C, McCarthy P, van Hoff J, Porter G.
    Journal: Pediatr Infect Dis J; 2008 Dec; 27(12):1116-8. PubMed ID: 18946363.
    Abstract:
    We report a case of Kawasaki disease with significant coronary artery aneurysms subsequently associated with reactive hemophagocytic lymphohistiocytosis in a young child with low T-cell perforin expression and NK-cell dysfunction. The patient was treated with a selective T-cell costimulation modulator in an effort to regulate T-cells. This case is unique for several reasons: (1) the severe degree of coronary artery aneurysms; (2) low T-cell perforin and NK-cell values; and (3) treatment with a selective T-cell costimulation modulator, none of which has been described in prior cases.
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