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Epstein-Barr virus infection and prognosis in haemophagocytic lymphohistiocytosis patients with underlying rheumatic diseases: a single-centre retrospective study


1, 2, 3, 4, 5, 6

 

  1. Department of Haematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China.
  2. Department of Haematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China.
  3. Department of Rheumatology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China.
  4. Department of Haematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China.
  5. Department of Haematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China.
  6. Department of Haematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China. wangzhao@ccmu.edu.cn

CER17280
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PMID: 40249053 [PubMed]

Received: 08/11/2023
Accepted : 27/02/2025
In Press: 18/04/2025

Abstract

OBJECTIVES:
Haemophagocytic lymphohistiocytosis (HLH) with underlying rheumatic diseases (rHLH) is a lethal disease, in which Epstein-Barr virus (EBV) infection is a causative factor. Whether EBV infection is associated with treatment response and prognosis of patients with rHLH remains unclear. This study explored the clinical features of patients with Epstein-Barr virus-positive rHLH.
METHODS:
In this retrospective study, we included 137-patients and divided them into EBV-negative (n=116) and EBV-positive (n=21) groups. We compared the clinical characteristics, treatment responses, and prognoses between the two groups. Propensity score matching (PSM) was used to match patients between groups. Kaplan-Meier analysis was used to elucidate the relationship between the EBV-infected cell type and prognosis.
RESULTS:
EBV-positive patients were more likely to have relapsed or refractory rHLH. The survival time of the EBV-negative group was significantly longer than that of the EBV-positive group (p=0.012). Further analysis of EBV-infected lymphocyte subsets revealed a significant decrease in survival in the NK and/or T lymphocyte groups compared to the other cell types (p<0.01).
CONCLUSIONS:
Patients with EBV-positive rHLH are more likely to experience relapse or refractoriness. For patients with rHLH, prompt testing of EBV-infected lymphocyte subsets should be performed upon EBV infection. An etoposide-based regimen is recommended for patients with EBV-positive rHLH, and rituximab may be effective in patients with refractory or relapsed rHLH with EBV-infected B lymphocytes. However, for patients with EBV-infected NK and/or T lymphocytes, treatment should be aligned with that for EBV-HLH.

DOI: https://doi.org/10.55563/clinexprheumatol/7l4od4

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