Abstract
BackgroundChronic active Epstein-Barr virus infection (CAEBV) disease is sometimes associated with an aggressive clinical course, such as hemophagocytic lymphohistiocytosis (HLH). To explore the risk factors and predict the risk of CAEBV infection progressing to HLH, a retrospective research study was conducted.MethodsWe retrospectively reviewed the medical records of 187 CAEBV-infected patients who were admitted to our center between January 2015 and December 2020. The patients were followed up until May 2021. The patients were divided into a progression-to-HLH group and a no-progression-to-HLH group. Demographic, clinical and laboratory data were collected for each patient.ResultsAmong the 121 CAEBV-infected patients who fulfilled the study's inclusion criteria, 48 (30.7%) patients did not progress to HLH, and 73 (60.3%) patients progressed to HLH. The median time from CAEBV infection to progression to HLH was 14 months, and the cumulative incidence rate of HLH increased as the duration of follow up increased (24.9, 47.3, 55.1, and 85.2% at 1, 3, 5, and 10 years, respectively). Multivariate analyses showed that the independent risk factors for CAEBV progression to HLH were plasma EBV-DNA load (OR = 3.239, 95% CI 1.219–8.603, P = 0.018), Platelet count (OR=0.991, 95%CI 0.985–0.998, P = 0.010), elevated alanine aminotransferase (OR=1.019, 95%CI 1.005–1.034, P = 0.009) and ≥2 of 3 lineages of cytopenia (OR=8.364, 95%CI 1.062–65.839, P = 0.044). The regression coefficients (β) from the multivariate logistic model were used to construct a model for estimating the risk of CAEBV infection progressing to HLH. The discriminatory ability of the model was good, and the area under the receiver operating characteristic (ROC) curve (AUC) was 0.925.Conclusionplasma EBV-DNA load, platelet count, elevated alanine aminotransferase and ≥ 2 of 3 lineages of cytopenia increase the risk of CAEBV infection progressing to HLH. A nomogram can be used to estimate the risk of CAEBV-infected patients progressing to HLH.
Highlights
Chronic active Epstein-Barr virus infection (CAEBV) disease is a lymphoproliferative disease associated with EBV infection that is characterized by chronic or recurrent infectious mononucleosislike symptoms, including fever, lymphadenopathy, hepatitis, splenomegaly or pancytopenia [1, 2]
Studies have shown that early Hematopoietic stem cell transplantation (HSCT) for patients in relatively good clinical condition may improve the prognosis of HSCT [12]; predicting the progression of CAEBV to hemophagocytic lymphohistiocytosis (HLH) is important for patients with a potential poor prognosis
The following data were collected for each patient: the age at onset, gender, clinical symptoms, interval time from clinical symptoms to diagnosis of CAEBV infection, EBV-infected lymphocyte subpopulations, EBV-DNA quantity in plasma, EBV-DNA quantity in peripheral blood mononuclear cells (PBMCs), splenomegaly, ≥2 of 3 lineages of cytopenia, platelet count (PLT), alanine aminotransferase (ALT), albumin (ALB), total bilirubin (TB), lactate dehydrogenase (LDH), decreased natural killer (NK) cell activity, hemophagocytosis in bone marrow, and presence of abnormal phenotypic cells in bone marrow
Summary
Chronic active Epstein-Barr virus infection (CAEBV) disease is a lymphoproliferative disease associated with EBV infection that is characterized by chronic or recurrent infectious mononucleosislike symptoms, including fever, lymphadenopathy, hepatitis, splenomegaly or pancytopenia [1, 2]. The clinical course of CAEBV disease is heterogeneous: some patients may survive for more than 10 years without effective treatment, whereas others progress rapidly to hemophagocytic lymphohistiocytosis (HLH), multiple organ failure, or leukemia/lymphoma within a few years [3–6]. A Japanese study showed that 24.4% of CAEBV-infected patients progressed to HLH [9]. HSCT treatment of CAEBV infection may result in life-threatening complications, patients with poor prognosis require aggressive treatment to reduce or eliminate EBV-infected cells. Studies have shown that early HSCT for patients in relatively good clinical condition may improve the prognosis of HSCT [12]; predicting the progression of CAEBV to HLH is important for patients with a potential poor prognosis. Chronic active Epstein-Barr virus infection (CAEBV) disease is sometimes associated with an aggressive clinical course, such as hemophagocytic lymphohistiocytosis (HLH). To explore the risk factors and predict the risk of CAEBV infection progressing to HLH, a retrospective research study was conducted
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