| Objective:To explore the clinical manifestations,complications,au xiliary examination,treatment and prognosis of children with chronic act ive Epstein-Barr virus infection(CAEBV),so as to increase cognition of CAEBV and provide reference for its diagnosis and treatment.Methods:The clinical data of thirteen children with CAEBV admi-tted to the Pediatric Hematology Ward of the Second Hospital of Hebei Medical University from January 1,2014 to December 31,2020 were retrospectively analyzed,including clinical manifestations,complications,auxiliary examination,treatment and prognosis.Results:1.Gender and age:There were thirteen children with CAEBV,including five boys and eight girls,ranging in age from three to fifteen years.The median age was seven years.Nine cases had a disease course of more than three years.2.Clinical features:All children presented with recurrent or persistent fever and splenomegaly.Twelve cases had hepatomegaly and nine cases had lymph node enlargement.Anemia was present in eleven cases.The other manifestations included skin rash in five cases,edema in three cases,swelling in the submaxillary area in two cases,diarrhea and convulsions in one case each.Complications occurred in 10children with CAEBV,including pneumonia in eight cases,coagulation disorders in eight cases,hypoalbuminemia in six cases,hemophagocytic syndrome in four cases,wider coronary artery in three cases,central nervous system lesions in three cases,sepsis in two cases,gastrointestinal bleeding,cellulitis,sjogren syndrome,polyarteritis,sudcutaneous panniculitis-like T-cell lymphoma in one case each.3.Laboratory test:EBV-DNA was positive in peripheral blood serum of the thirteen cases,and the copy number was from10~3to 10~9per millilitre.One case had positive EBV-DNA in cerebrospinal fluid.Eleven cases were tested for Epstein-Barr virus antibody,and all of them had positive VCA-Ig G.Ten cases had positive NA-Ig G and one case had weak positive NA-Ig G.Five cases had positive EA-Ig G.One child showed positive anti-SSA-60K and anti-SSA-52KD by autoantibody test.Twelve children underwent bone marrow cytology examination,and seven cases showed atypical lymphocytes or juvenile cells,four cases showed hemophagocytes.Six cases underwent tissue biopsy,including two cases with reactive hyperplasia of lymph nodes,one with extensive necrosis of lymph nodes,two with lymphoproliferative disease,and one with sudcutaneous panniculitis-like T-cell lymphoma.4.Therapies:The treatment included antiviral drugs(13/13),immunosuppressant(11/13),immunoglobulin(3/13),splenectomy(1/13),infusion of Epstein-Barr virus specific cytotoxic T lymphocyte(1/13),and allogeneic hematopoietic stem cell transplantation(1/13).5.Survival situation:Among the thirteen cases,ten cases died and three cases survived,with a mortality rate of 76.9%.Five cases died during hospitalization in our hospital,and the other five cases died after being transferred to other hospitals for treatment.Conclusions:1.Chronic active Epstein-Barr virus infection(CAEBV)was rare in clinic.It was difficult to make a clear diagnosis at initial diagnosis and prone to occur life-threatening complications.So it had a high fatality rate and poor prognosis.2.It had various manifestations.The main manifestation was infectious mononucleosis-like symptoms,accompanied by abnormal increase in viral load of EBV-DNA in peripheral blood.3.If patients have recurrent or persistent fever and hepatosplenomegaly of lymph nodes,the possibility of CAEBV should be considered and the diagnosis should be made as soon as possible based on the medical history,the viral load of EBV-DNA,the EBV antibody profile,tissue biopsy,etc.4.At present,allogeneic hematopoietic stem cell transplantation is considered to be the only radical cure. |
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