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KMID : 1100720190390050430
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Annals of Laboratory Medicine
2019 Volume.39 No. 5 p.430 ~ p.437
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Flow Cytometric Analysis of T Cells in Hemophagocytic Lymphohistiocytosis
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Park Min-Seung
Yoo In-Young
Kim Hee-Jin
Kim Sun-Hee
Kim Seok-Jin
Cho Duck
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Abstract
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Background: T cell immunophenotypes in patients with hemophagocytic lymphohistiocytosis (HLH) have been described. Downregulation of CD5 or CD7 on T cells has been reported in patients with Epstein-Barr virus (EBV)-positive HLH. As the utility of T cell immunophenotypes as an adjunctive diagnostic or a prognostic marker for HLH has not been evaluated, we analyzed T cell immunophenotypes in HLH patients for this purpose.
Methods: We classified 45 HLH patients into three subgroups: EBV-positive HLH (N=27), EBV-negative secondary HLH (N=15), and familial HLH (N=3). We retrospectively characterized downregulation patterns of CD5 or CD7 on activated T cells, using flow cytometry. Overall survival was estimated using Kaplan-Meier curves and compared using the log-rank test.
Results: An aberrant immunophenotype, including CD5 and/or CD7 downregulation on T cells, was observed in 55.6% (15/27) of the EBV-positive HLH patients and 100% of the familial HLH (3/3). Only one (1/15, 6.7%) patient with EBV-negative secondary HLH showed an aberrant loss of CD7 antigen on CD8+ T cells. The presence of an aberrant T cell immunophenotype was not related to overall survival in EBV-positive HLH and EBV-negative secondary HLH patients.
Conclusions: An aberrant T cell immunophenotype may assist in discriminating EBV-negative secondary HLH and EBV-positive HLH. However, it may not be useful as a prognostic marker.
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KEYWORD
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Epstein-Barr virus, Familial, Hemophagocytic lymphohistiocytosis, Flow cytometry, T cell, Overall survival
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