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Clinical significance of clonality and Epstein‐Barr virus infection in adult patients with hemophagocytic lymphohistiocytosis
Author(s) -
Ahn JaeSook,
Rew SungYoon,
Shin MyungGeun,
Kim HyeRan,
Yang DeokHwan,
Cho Duck,
Kim SooHyun,
Bae Soo Young,
Lee Se Ryeon,
Kim YeoKyeoung,
Kim HyeoungJoon,
Lee JeJung
Publication year - 2010
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.21795
Subject(s) - hemophagocytic lymphohistiocytosis , epstein–barr virus , immunology , viral load , virus , polymerase chain reaction , clinical significance , immunoglobulin heavy chain , biology , gammaherpesvirinae , herpesviridae , antibody , medicine , viral disease , disease , gene , genetics
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.