Ann Lab Med 2019; 39(5): 430-437  https://doi.org/10.3343/alm.2019.39.5.430
Flow Cytometric Analysis of T Cells in Hemophagocytic Lymphohistiocytosis
Min-Seung Park , M.D.1,*, In Young Yoo , M.D.1,*, Hee Jin Kim , M.D.1, Sun-Hee Kim , M.D.1, Seok Jin Kim , M.D.2, and Duck Cho , M.D.1
1Department of Laboratory Medicine and Genetics and 2Division of Hematology and Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
Corresponding author: Duck Cho, M.D
Department of Laboratory Medicine and Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 06351, Korea
Tel: +82-2-3410-2403, Fax: +82-2-3410-2719, E-mail: duck.cho@samsung.com
Seok Jin Kim, M.D.
Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 06351, Korea
Tel: +82-2-3410-1766, Fax: +82-2-3410-1754, E-mail: kstwoh@skku.edu
*These authors contributed equally to this work.
Received: November 24, 2018; Revised: January 13, 2019; Accepted: April 15, 2019; Published online: September 1, 2019.
© Korean Society for Laboratory Medicine. All rights reserved.

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract
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.
Keywords: Epstein-Barr virus, Familial, Hemophagocytic lymphohistiocytosis, Flow cytometry, T cell, Overall survival



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