scholarly journals Clinical analysis of chronic active EBV infection with coronary artery dilatation and a matched case–control study

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Ang Wei ◽  
Honghao Ma ◽  
Liping Zhang ◽  
Zhigang Li ◽  
Yitong Guan ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Serum Ferritin ◽  
Epstein Barr Virus ◽  
Epstein Barr Virus Infection ◽  
Hematopoietic Stem ◽  
Barr Virus ◽  
Coronary Artery Dilatation ◽  
Tnf Α ◽  
Epstein Barr

Abstract Objective To investigate the clinical characteristics, treatment, prognosis and risk factors for chronic active Epstein–Barr Virus infection (CAEBV) associated with coronary artery dilatation (CAD) in children. Methods Children with CAEBV associated with CAD hospitalized at Beijing Children’s Hospital, Capital Medical University from March 2016 to December 2019 were analyzed. Children with CAEBV without CAD were selected as the control group and matched by sex, age, treatment and admission time. The clinical manifestations, laboratory and ultrasound examinations, treatment and prognosis of the children were collected in both groups. Results There were 10 children with CAEBV combined with CAD, including 6 males and 4 females, accounting for 8.9% (10/112) of CAEBV patients in the same period, with an onset age of 6.05 (2.8–14.3) years. The median follow-up time was 20 (6–48) months. All the patients had high copies of EBV-DNA in whole blood [1.18 × 107 (1.90 × 105–3.96 × 107) copies/mL] and plasma [1.81 × 104 (1.54 × 103–1.76 × 106) copies/mL], and all biopsy samples (bone marrow, lymph nodes or liver) were all positive for Epstein–Barr virus-encoded small RNA. Among the 10 children, 8 had bilateral CAD, and 2 patients had unilateral CAD. After diagnosis, 7 children were treated with L-DEP chemotherapy in our hospital. After chemotherapy, four patients underwent allogeneic hematopoietic stem cell transplantation (HSCT). The others were waiting for HSCT. At the time of the last patients follow up record, the CAD had returned to normal in 3 patients, and the time from the diagnosis of CAD to recovery was 21 (18–68) days. LDH, serum ferritin, TNF-α and IL-10 levels were statistically significantly different between the two groups (P = 0.009, 0.008, 0.026 and 0.030). There were no significant differences in survival rate between the two groups (P = 0.416). Conclusion The incidence of CAEBV with CAD was low. CAEBV with CAD did not influence the prognosis. Patients who had high LDH, serum ferritin, TNF-α, and IL-10 levels early in their illness were more likely to develop CAD.

scholarly journals Clinical Analysis of Chronic Active EBV Infection with Coronary Artery Dilatation and Matched Case-control Study

2020 ◽  
Author(s):  
Ang Wei ◽  
Honghao Ma ◽  
Liping Zhang ◽  
Zhigang Li ◽  
Yitong Guan ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Serum Ferritin ◽  
Clinical Manifestations ◽  
Control Group ◽  
Hematopoietic Stem ◽  
Ebv Infection ◽  
Coronary Artery Dilatation ◽  
Tnf Α ◽  
Significant Difference

Abstract Objective To investigate the clinical characteristics, treatment, prognosis, and risk factors of chronic active EBV infection (CAEBV) associated with coronary artery dilatation (CAD) in children.Methods Children with CAEBV associated with CAD hospitalized in Beijing Children’s Hospital, Capital Medical University, from March 2016 to December 2019 were analyzed. At the same time, children with CAEBV without CAD were selected as the control group, matched by sex, age, treatment and admission time. The clinical manifestations, laboratory and ultrasonic examinations, treatment and prognosis of the children were collected in both groups.Results There were 10 children with CAEBV combined with CAD, accounting for 8.9% (10/112) of CAEBV patients at the same period, which including 6 males and 4 females, with onset age of 6.05 (2.8-14.3) years. The median follow-up time was 20 (6-48) months. All the patients had high copies of EBV-DNA in whole blood 1.18x107（1.90x105-3.96x107）copies/mL and plasma 1.81x104（1.54x103-1.76x106）copies/mL, and the Epstein-Barr virus encoded small RNA in biopsy was all positive. Among the 10 children, 8 had bilateral CAD, with 2 patients unilateral. After diagnosis, 7 children were treated with L-DEP chemotherapy in our hospital. After chemotherapy, four patients accepted allo-genetic Hematopoietic Stem Cell Transplantation (HSCT). The others were waiting for HSCT. By the end of the follow-up, CAD had returned to normal in 3 patients, and the time from diagnosis of CAD to recovery was 21 (18-68) d. The level of LDH, serum ferritin, TNF-α and IL-10 had statistically significant difference between the two groups (P=0.009, 0.008, 0.026 and 0.030). There were no significant differences in survival rate between the two groups (P=0.416).Conclusion The incidence of CAEBV with CAD was low. CAEBV with CAD did not influence the prognosis. Patients with CAEBV had high LDH, serum ferritin, TNF-α and IL-10 in the early onset were prone to have CAD.

scholarly journals Chronic active Epstein–Barr virus infection manifesting as coronary artery aneurysm and uveitis

2020 ◽  
Vol 17 (1) ◽  
Author(s):  
Haijuan Xiao ◽  
Bing Hu ◽  
Rongmu Luo ◽  
Huili Hu ◽  
Junmei Zhang ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Peripheral Blood ◽  
Epstein Barr Virus ◽  
Coronary Artery Aneurysm ◽  
Artery Aneurysm ◽  
Epstein Barr Virus Infection ◽  
Hematopoietic Stem ◽  
Barr Virus ◽  
Ebv Dna ◽  
Epstein Barr

Abstract Background Chronic active Epstein–Barr virus (CAEBV) infection is a type of lymphoproliferative disorder characterized by chronic or recurrent infectious mononucleosis (IM)-like symptoms, which can have less-frequent clinical presentations. The prognosis of CAEBV is poor, and hematopoietic stem cell transplantation (HSCT) has been shown to be the only potentially effective treatment. In this article, we present a special CAEBV case of a patient who had no typical IM-like symptoms at the early stage, but manifested with severe and progressive coronary artery aneurysm (CAA), abdominal aortic lesions, and severe uveitis. These manifestations were uncommon features and could only be blocked by HSCT. Case presentation A 4-year-old girl with no special medical history complained of decreased vision for 10 months and cough after physical activities for three months. The blurred vision grew rapidly worse within one month, until only light perception remained. She was diagnosed with uveitis and cataract, and received prednisone and ciclosporin A treatment. However, her vision did not improve. Physical examination showed slight hepatosplenomegaly. Ultrasonic cardiogram showed bilateral CAA (5.0 mm and 5.7 mm for inner diameters), and abdominal CT scan revealed a thickened aortic wall, as well as stenosis and dilation of the segmental abdominal aorta. Other significant findings were increased EBV-DNA (3.29 × 104 copies/mL) from peripheral blood, positive EBV antibodies (EBV-CA-IgG, EBV-EA-IgA, and EBV-NA-IgG), and positive EBV-encoded small RNAs found by bone marrow biopsy. Based on her clinical manifestations and evidence for EBV infection, we diagnosed CAEBV. She received allogeneic HSCT, and the cataract operation was performed after HSCT. EBV-DNA could not be detected in peripheral blood after HSCT. Her CAAs did not progress, and uveitis was well controlled. Her vision recovered gradually over the 3 years after HSCT. Conclusions We present a rare CAEBV case of a patient who suffered from uncommon and severe cardiovascular and ocular involvement that was relieved by HSCT. Therefore, early recognition and diagnosis of CAEBV are of vital importance to improve its prognosis. In summary, this atypical CAEBV case could help us recognize similar cases more easily, make the right diagnosis as early as possible, and deliver proper and timely treatment.

scholarly journals Outcome of L-DEP Regimen for Treatment of Pediatric Chronic Active Epstein-Barr Virus Infection

2021 ◽  
Author(s):  
Honghao Ma ◽  
Liping Zhang ◽  
Ang Wei ◽  
Jun Yang ◽  
Dong Wang ◽  
...  
Keyword(s):  
Virus Infection ◽  
Clinical Response ◽  
Virological Response ◽  
Epstein Barr Virus ◽  
Survival Rates ◽  
Epstein Barr Virus Infection ◽  
Hematopoietic Stem ◽  
Barr Virus ◽  
Epstein Barr ◽  
Barr Virus Infection

Abstract Purpose We intended to investigate the clinical features of pediatric patients with chronic active Epstein-Barr virus infection (CAEBV) and effectiveness of the L-DEP regimen before HSCT (hematopoietic stem cell transplantation).Methods A retrospective analysis was performed on 35 patients with CAEBV at Beijing Children’s Hospital from January 2016 to January 2020. The efficacy and adverse events of the L-DEP regimen were evaluated.Results The median age of 35 patients was 7.0 years (range 2.5-17.5 years). 28 patients achieved clinical response (80.0%, 22 in clinical CR, 6 in clinical PR) after L-DEP. In terms of virological response, 7 patients (20%) were assessed as virological CR and 23 patients (65.7%) were virological PR. Finally, 29 patients underwent allo-HSCT. Median survival time was 18 months (2-50 months). The 3-year overall survival rates in patients treated with chemotherapy only (n=6), chemotherapy followed by HSCT (n=25) were 33.3% and 75.4%, respectively. After L-DEP 1st treatment, the amount of EBV-DNA loads in blood and plasma were significantly reduced than that before chemotherapy (median: 4.29×105 vs. 1.84×106, Mann-Whitney U：P=0.0004; 5.00×102 vs. 3.17×103, Mann-Whitney U：P=0.003). And, compared with liver and spleen size before chemotherapy, the size of liver and spleen shrank significantly after L-DEP 2nd (median 3.8cm vs. 1.9cm, P=0.003; 3.8cm vs. 0cm, P＜0.008). In addition, after L-DEP treatment, there was no difference in clinical or virological response rate whether HLH was accompanied or not (clinical response: 77.3% vs. 84.6%：P=0.689; virological response: 90.9% vs. 76.9%, P=0.337). Conclusion L-DEP regimen is an effective therapy in treating CAEBV for bridging to allo-HSCT.

scholarly journals Rituximab in the Treatment of Epstein-Barr Virus Infection after hematopoietic stem cell Transplantation in Children

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5892-5892
Author(s):  
Zhiyong Peng ◽  
Chunfu Li ◽  
Yuelin He
Keyword(s):  
Stem Cell ◽  
Epstein Barr Virus ◽  
Epstein Barr Virus Infection ◽  
Hematopoietic Stem ◽  
Viral Loads ◽  
Barr Virus ◽  
Ebv Infection ◽  
Infection Disease ◽  
Epstein Barr ◽  
Barr Virus Infection

Abstract OBJECTIVE: To investigate the efficacy and safety of Rituximab on Epstein-Barr virus infection disease after allogeneic hematopoietic stem-cell transplantation (allo-HSCT) in children. METHODS: A retrospective analysis was performed based on clinical data of 25 children with median age of 6 years, (3~14 years) diagnosed as EBV infection and received Rituximab from Jan. 2012 to Jun. 2014 in our center. Of them, 3 patients were diagnosed as post-transplant lymphoproliferative disorders (PTLD). All patients received Rituximab at dose of 375 mg/m2 once a week for 4 sequence weeks or discontinued therapy when EBV viral loads was <500 copies/mL two times consecutively. The complete remission (CR) was defined as EBV viral loads <500 copies/mL consecutively at least two time and without any related clinical symptoms. Side effects during infusion were evaluated by Common Terminology Criteria for Adverse Events. RESULTS: The median EBV-infection time was on day 70 (18~200) after HSCT. Each of patients received a median of 2 (1~4) infusions. There were no severe side effects during the infusion of Rituximab. The cumulative complete remission (CR) rate were 88% in total. The CR rate was 56.0±9.9 %, 80.0±8.0%, 84.0±7.3%, and 88.0±6.5%, respectively, in 1st, 2nd, 3rd, and 4th week. Two patients died, one due to infections and the other died of hepatitis while receiving therapy. The CR rate was 100% in patients with PTLD (3/3). CONCLUSIONS: These data suggest Rituximab is safe and effective when treating children with EBV infection disease after allo-HSCT. Disclosures No relevant conflicts of interest to declare.

Sign in / Sign up

Export Citation Format

Share Document