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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.

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CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Brief report

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

From the Division of Pediatric Hematology, Oncology, Blood, and Marrow Transplant, Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, WI; the Division of Pediatric Hematology, Oncology, Blood, and Marrow Transplant, University of Iowa Hospitals and Clinics, Iowa City, IA; and the Division of Pediatric Infectious Disease, University of Iowa Hospitals and Clinics, Iowa City, IA.

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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