Journal article
Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8
Blood, Vol.106(4), pp.1203-1206
08/15/2005
DOI: 10.1182/blood-2005-03-0950
PMCID: PMC1895204
PMID: 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.
Details
- Title: Subtitle
- Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8
- Creators
- William J Grossman - 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, IAMohammed Radhi - 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, IADennis Schauer - 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, IAErick Gerday - 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, IACharles Grose - 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, IAFrederick 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
- Resource Type
- Journal article
- Publication Details
- Blood, Vol.106(4), pp.1203-1206
- Publisher
- The American Society of Hematology
- DOI
- 10.1182/blood-2005-03-0950
- PMID
- 15840696
- PMCID
- PMC1895204
- ISSN
- 0006-4971
- eISSN
- 1528-0020
- Language
- English
- Date published
- 08/15/2005
- Academic Unit
- Stead Family Department of Pediatrics; Infectious Disease (Pediatrics)
- Record Identifier
- 9984093344202771
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