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  • Title: Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8.
    Author: Grossman WJ, Radhi M, Schauer D, Gerday E, Grose C, Goldman FD.
    Journal: Blood; 2005 Aug 15; 106(4):1203-6. PubMed ID: 15840696.
    Abstract:
    Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of immune dysregulation, characterized by end-organ damage from lymphocytic infiltration and macrophage activation. All known mutations associated with the HLH occur in genes critical in the perforin-granzyme pathway. Herein, we report HLH occurring in 2 female triplet infants who also had associated human herpesvirus type 8 (HHV-8) infections. The subjects had identical novel compound-heterozygous mutations in the Perforin alleles, resulting in undetectable perforin expression and NK-cell cytotoxicity. Both infants also had evidence of infection with HHV-8. These reports are, to our knowledge, the first cases of HLH in triplets and the first reported cases of HHV-8 infection associated with HLH in non-renal transplant and non-HIV-infected subjects.
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