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

被引：65

作者：

Ahn, Jae-Sook ^{[1
]}

Rew, Sung-Yoon ^{[1
]}

Shin, Myung-Geun ^{[2
,3
]}

Kim, Hye-Ran ^{[3
]}

Yang, Deok-Hwan ^{[1
]}

Cho, Duck ^{[2
]}

Kim, Soo-Hyun ^{[2
]}

Bae, Soo Young ^{[1
]}

Lee, Se Ryeon ^{[4
]}

Kim, Yeo-Kyeoung ^{[1
]}

Kim, Hyeoung-Joon ^{[1
]}

Lee, Je-Jung ^{[1
,3
]}

机构：

[1] Chonnam Natl Univ, Dept Hematol Oncol, Hwasun Hosp, Hwasungun 519809, Jeollanamdo, South Korea

[2] Chonnam Natl Univ, Dept Lab Med, Hwasun Hosp, Hwasungun 519809, Jeollanamdo, South Korea

[3] Chonnam Natl Univ, Sch Med, Brain Korea Project 21, Ctr Biomed Human Resources, Kwangju, South Korea

[4] Korea Univ, Div Hematol Oncol, Med Ctr, Seoul, South Korea

来源：

AMERICAN JOURNAL OF HEMATOLOGY | 2010年 / 85卷 / 09期

关键词：

CONCERTED ACTION BMH4-CT98-3936; T-CELLS; DISEASE; LYMPHOPROLIFERATIONS; PATHOGENESIS; MANAGEMENT; LYMPHOMA; JAPAN;

D O I：

10.1002/ajh.21795

中图分类号：

R5 [内科学];

学科分类号：

100201 [内科学];

摘要：

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.

引用

页码：719 / 722

页数：4

共 17 条

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