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Identifying Dysregulated Genes Induced by Kaposi's Sarcoma-associated Herpesvirus (KSHV)
07:02

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Published on: September 14, 2010

Kawasaki disease associated with reactive hemophagocytic lymphohistiocytosis

Christy Cummings1, Paul McCarthy, Jack van Hoff

  • 1Department of Pediatrics, Yale-New Haven Children's Hospital, 20 York Street, New Haven, CT 06511, USA. Christy.Cummings@yale.edu

The Pediatric Infectious Disease Journal
|October 24, 2008
PubMed

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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