title: Clinical significance of clonality and Epstein-Barr virus infection in adult patients with hemophagocytic lymphohistiocytosis.
abstract: We assessed the clinical significance of T or B cell clonality and Epstein-Barr virus (EBV) infection in adult patients with hemophagocytic lymphohistiocytosis (HLH) to identify factors related to prognosis. A total of 30 adult patients with diagnosed HLH were included in the study. In all patients, EBV-DNA in peripheral blood was examined by quantitative real-time polymerase chain reaction and bone marrow cells were examined for clonal rearrangement of T cell receptor gamma(TCRG) and immunoglobulin heavy chain (IGH) genes. TCRG clones were detected in 10 patients (33.3%) and IGH clones were detected in 8 patients (26.7%). We found no correlation between clonality and patient outcome. The patients less than 1,000 copies (mL)21 of EBVDNA showed a significantly higher clinical response (P 5 0.008) and longer overall survival (P 5 0.01) than those with high viral load of EBV-DNA. Our results suggest that TCRG and IGH rearrangement do not have any clinical significance in adult patients with HLH, but that high viral load of EBV-DNA may be a risk factor for poor outcomes. In HLH, high viral load of EBV-DNA should thus suggest a prompt approach with aggressive therapeutic interventions.
authors.forename: Jae-Sook
authors.initials: JS
authors.lastname: Ahn
authors.forename: Sung-Yoon
authors.initials: SY
authors.lastname: Rew
authors.forename: Myung-Geun
authors.initials: MG
authors.lastname: Shin
authors.forename: Hye-Ran
authors.initials: HR
authors.lastname: Kim
authors.forename: Deok-Hwan
authors.initials: DH
authors.lastname: Yang
authors.forename: Duck
authors.initials: D
authors.lastname: Cho
authors.forename: Soo-Hyun
authors.initials: SH
authors.lastname: Kim
authors.forename: Soo Young
authors.initials: SY
authors.lastname: Bae
authors.forename: Se Ryeon
authors.initials: SR
authors.lastname: Lee
authors.forename: Yeo-Kyeoung
authors.initials: YK
authors.lastname: Kim
authors.forename: Hyeoung-Joon
authors.initials: HJ
authors.lastname: Kim
authors.forename: Je-Jung
authors.initials: JJ
authors.lastname: Lee
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001402:B-Lymphocytes / Q000378:metabolism / Q000821:virology*; D004279:DNA, Viral / Q000097:blood*; D020031:Epstein-Barr Virus Infections / Q000097:blood / Q000209:etiology / Q000401:mortality; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000378:metabolism; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000150:complications / Q000401:mortality / Q000821:virology; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction / Q000379:methods; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000378:metabolism; D012307:Risk Factors; D015996:Survival Rate; D013601:T-Lymphocytes / Q000378:metabolism / Q000821:virology*; D019562:Viral Load
chemical_list: D004279:DNA, Viral; D007143:Immunoglobulin Heavy Chains; D016692:Receptors, Antigen, T-Cell, gamma-delta