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Blood, 15 August 2005, Vol. 106, No. 4, pp. 1203-1206. Prepublished online as a Blood First Edition Paper on April 19, 2005; DOI 10.1182/blood-2005-03-0950. This Article Full Text (PDF) All Versions of this Article: 2005-03-0950v1 106/4/1203    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Grossman, W. J Articles by Goldman, F. D Search for Related Content PubMed PubMed Citation Articles by Grossman, W. J Articles by Goldman, F. D Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted March 8, 2005 Accepted April 7, 2005 Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8 William J Grossman*, Mohammed Radhi, Dennis Schauer, Erick Gerday, Charles Grose, and Frederick D Goldman Division of Pediatric Hematology/Oncology/Blood & Marrow Transplant, Medical College of Wisconsin/Children's Hospital of Wisconsin, Milwaukee, WI, USA Division of Pediatric Hematology/Oncology/Blood & Marrow Transplant, University of Iowa Hospitals and Clinics, Iowa City, IA, USA Division of Pediatric Infectious Disease, University of Iowa Hospitals and Clinics, Iowa City, IA, USA * Corresponding author; email: wgrossma{at}mcw.edu. 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 two 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. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: Y. T. Bryceson, E. Rudd, C. Zheng, J. Edner, D. Ma, S. M. Wood, A. G. Bechensteen, J. J. Boelens, T. Celkan, R. A. Farah, et al. Defective cytotoxic lymphocyte degranulation in syntaxin-11 deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients Blood, September 15, 2007; 110(6): 1906 - 1915. [Abstract] [Full Text] [PDF]

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