title: Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Los Angeles County.
abstract: Data on Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH) in adults in the United States remain very limited. A cluster of four cases of EBV-HLH was observed in a 4-month period at a tertiary center in Los Angeles County (LA County) and the clinical and molecular characteristics identified in these cases are being described. EBV typing, immunophenotypic and molecular genetic studies were performed. Diagnostic criteria that may be used to identify EBV as a cause of HLH in adults are also being suggested. Finally, the crude incidence rate for HLH in LA County was determined and was compared to the worldwide crude incidence rate for HLH. The cases each occurred in young male adult residents of California and were associated with evidence of EBV reactivation and ferritin levels of >20,000 µg/L. A higher rate of cases of EBV-HLH in 2010 was found at UCLA Medical Center than for 2007-2009 (4.9/10,000 hospital discharges vs. 0.14/10,000 hospital discharges, respectively; P = 0.0017). The cases were associated with EBV type 1, and the insertion of the codon CTC (leucine) was found in numerous of the EBNA-2 gene sequences. The annual incidence of secondary, non-familial HLH was estimated to be 0.9 cases per million persons >15 years of age in LA County. Although EBV-HLH is a rare disease, the incidence in adults in Western countries may be underestimated.
authors.affiliation: Division of Infectious Diseases, Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA. tkelesidis@mednet.ucla.edu
authors.forename: Theodoros
authors.initials: T
authors.lastname: Kelesidis
authors.forename: Romney
authors.initials: R
authors.lastname: Humphries
authors.forename: Dawn
authors.initials: D
authors.lastname: Terashita
authors.forename: Shahrooz
authors.initials: S
authors.lastname: Eshaghian
authors.forename: Mary C
authors.initials: MC
authors.lastname: Territo
authors.forename: Jonathan
authors.initials: J
authors.lastname: Said
authors.forename: Michael
authors.initials: M
authors.lastname: Lewinski
authors.forename: Judith S
authors.initials: JS
authors.lastname: Currier
authors.forename: David
authors.initials: D
authors.lastname: Pegues
mesh_terms: D000328:Adult; D000595:Amino Acid Sequence; D004279:DNA, Viral / Q000032:analysis; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000821:virology; D019309:Epstein-Barr Virus Nuclear Antigens / Q000235:genetics; D004854:Herpesvirus 4, Human / Q000145:classification / Q000235:genetics*; D006801:Humans; D015141:Los Angeles / Q000453:epidemiology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D008297:Male; D008969:Molecular Sequence Data; D017422:Sequence Analysis, DNA; D014763:Viral Matrix Proteins / Q000235:genetics; D014764:Viral Proteins / Q000235:genetics; D055815:Young Adult
chemical_list: D004279:DNA, Viral; C402641:EBNA-2 protein, Human herpesvirus 4; C037662:EBV-associated membrane antigen, Epstein-Barr virus; D019309:Epstein-Barr Virus Nuclear Antigens; D014763:Viral Matrix Proteins; D014764:Viral Proteins