Ang Wei1, Honghao Ma1, Liping Zhang1, Zhigang Li2, Yitong Guan1, Qing Zhang2, Dong Wang1, Hongyun Lian1, Rui Zhang3,4, Tianyou Wang5,6. 1. Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, People's Republic of China. 2. Hematology and Oncology Laboratory, Beijing Pediatric Research Institute, Beijing Children's Hospital Affiliated with Capital Medical University; National Center for Children's Health; Beijing Key Laboratory of Pediatric Hematology Oncology, Key Laboratory of Major Diseases in Children; Ministry of Education, National Key Discipline of Pediatrics, Beijing, 100045, People's Republic of China. 3. Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, People's Republic of China. ruizh1973@126.com. 4. Department of Hematology and Oncology, Beijing Children's Hospital, Capital Medical University, Nanlishi Road No. 56, Xicheng District, Beijing, 100045, People's Republic of China. ruizh1973@126.com. 5. Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, People's Republic of China. wangtianyou@bch.com.cn. 6. Department of Hematology and Oncology, Beijing Children's Hospital, Capital Medical University, Nanlishi Road No. 56, Xicheng District, Beijing, 100045, People's Republic of China. wangtianyou@bch.com.cn.
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.
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.
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