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Clinical significance of clonality and Epstein‐Barr virus infection in adult patients with hemophagocytic lymphohistiocytosis

2010; Wiley; Volume: 85; Issue: 9 Linguagem: Inglês

10.1002/ajh.21795

1096-8652

Jae‐Sook Ahn, Sung‐Yoon Rew, Myung‐Geun Shin, Hye‐Ran Kim, Deok‐Hwan Yang, Duck Cho, Soo‐Hyun Kim, Soo Young Bae, Se Ryeon Lee, Yeo‐Kyeoung Kim, Hyeoung‐Joon Kim, Je‐Jung Lee,

Immune Cell Function and Interaction

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.