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

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

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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)-1 of EBV-DNA showed a significantly higher clinical response (P = 0.008) and longer overall survival (P = 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.

Original languageEnglish
Pages (from-to)719-722
Number of pages4
JournalAmerican Journal of Hematology
Volume85
Issue number9
DOIs
Publication statusPublished - 2010 Sep

ASJC Scopus subject areas

  • Hematology

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    Ahn, J. S., Rew, S. Y., Shin, M. G., Kim, H. R., Yang, D. H., Cho, D., Kim, S. H., Bae, S. Y., Lee, S. R., Kim, Y. K., Kim, H. J., & Lee, J. J. (2010). Clinical significance of clonality and Epstein-Barr virus infection in adult patients with hemophagocytic lymphohistiocytosis. American Journal of Hematology, 85(9), 719-722. https://doi.org/10.1002/ajh.21795