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. J. Med. Virol. 84:777–785, 2012. © 2012 Wiley Periodicals, Inc.

Hemophagocytic lymphohistiocytosis: review of etiologies and management

“…59 While fulminant infectious mononucleosis may overlap with EBV-associated HLH, higher viral loads are seen in EBV-associated HLH. 54 While not as commonly reported nor as well defined as EBV, many other viruses may be associated with HLH. These can be best organized by DNA or RNA (ribonucleic acid) and, subsequently, by virus family, as listed in Table 2.…”

“…These studies have demonstrated that no single sub-strain of EBV has been linked to HLH as of yet. [54][55][56] It appears that new infection as well as reactivation of latent EBV infection may predispose individuals to HLH. Quantitative determination of EBV genome copy numbers in peripheral blood may be useful in predicting prognosis and effectiveness of therapy.…”

Young Male with Secondary Acute Hemophagocytic Lymphohistiocytosis

“…The list of possible etiologies for secondary HLH is long and includes infections (mainly EBV, herpes simplex virus, cytomegalovirus, avian influenza) [2][3][4] , rheumatologic diseases (rheumatoid arthritis, systemic lupus erythemtosus, kawasaki disease, adult onset still's disease ) [5,6] , malignacy (natural killer cell leukemia, peripheral T cell lymphoma) [7,8] , acquired immune deficiency states (after organ transplants), and drugs [9] .…”

Haemophagocytic lymphohistiocytosis presenting as liver failure following Epstein-Barr and prior hepatitis A infections

“…EBV infection has been the most commonly reported viral trigger for acquired HLH and the clinical characteristics vary from mild-to-severe; for its diagnosis, the patient must fulfil the HLH diagnostic criteria described in box 1. Antiviral therapy, intravenous immunoglobulin, rituximab and alemtuzumab have been used for patients who relapse, with haematopoietic stem cell transplantation if other therapies fail 7 13 22. There have also been a few reported cases of HLH after HAV infection.…”