Clinical analysis of 19 cases of chronic active Epstein-Barr virus infection in children

Abstract

Objective To analyze the clinical characteristics and prognosis of children with chronic active Epstein-Barr virus infection (CAEBV). Methods The clinical characteristics, laboratory test findings, treatment and prognosis of 19 cases of children with CAEBV during February 2011 to August 2016 in Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science Technology were analyzed retrospectively. Results The major signs and symptoms of children with CAEBV were fever (18 cases), hepatomegaly (17 cases), splenomegaly (17 cases), lymphadenopathy (11 cases) and blister-like rash (5 cases). Blood routine and liver function results showed leukopenia (11 cases) and anemia (11 cases), neutropenia (8 cases), thrombocytopenia (2 cases), elevated alanine aminotransferase (11 cases), elevated aspartate aminotransferase (10 cases). EBV DNA load increased, viral capsid antigen(VCA)-IgG and nuclear antigen (NA)-IgG were positive in peripheral blood of all cases, and early antigen(EA)-IgG was positive in 8 cases.The results of lymphocyte subsets in 18 cases showed that the proportion of natural killer cell(NK cell) significantly increased in 7 cases, and 1 case was slightly elevated.The proportion of T cells significantly increased in 4 cases, and 2 cases were slightly elevated.The proportion of B cells significantly increased in 1 case; but the results of lymphocyte subsets had no obvious abnormalities in other 3 cases.Genetic testing of 6 cases showed that 3 cases were negative, 1 case with a homozygous mutation in the X-linked inhibitor of apoptosis protein gene(XIAP gene), and 2 cases had heterozygous mutation (the meaning of mutation was unclear in 1 case). Three out of 11 cases had hemophagocytic syndrome by bone marrow examination, and 1 case out of 4 cases had lymphoma by lymph node biopsy.All of 19 cases of CAEBV had complications, including 6 cases of pneumonia, 3 cases of hemo-phagocytic lymphoid Histiocytosis (HLH), 3 cases of thrush, and lymphoma, intestinal obstruction, intestinal perforation and intestinal bleeding, cholecystitis, multiple stones in intrahepatic bile duct, autoimmune hepatitis, purpura, perianal abscess, malnutrition and hypoalbuminemia respective in 1 case respectively.Nineteen children with CAEBV received different treatment based on patients′ condition, 3 cases discontinued, 8 cases survived (1 case diagnosed recently 1 year and 1 case underwent successfully, living for 5 years now), 8 patients died, and the mortality rate was 50.0% (8/16 cases). Conclusions The main clinical characteristics of children with CAEBV are fever, hepatosplenomegaly, lymphadenopathy, skin rash, blood system involvement and elevated transaminase; CAEBV is prone to pneumonia, then HLH and other complications may appear.EBV DNA load increases in peripheral blood cells as an important evidence of viral attack; lymphocyte subset of peripheral blood is significant for typing of CAEBV, and most cases belong to NK cell type and T cell type probably; lymph node biopsy and bone marrow examination are extremely important for identifying whether it is prone to lymphoma or HLH.The treatment should be based on the condition of individual condition to select immunosuppression and/or chemotherapy, and hematopoietic stem cell transplantation is best choice to improve the prognosis at present. Key words: Chronic active Epstein-Barr virus infection; Clinical characteristics; Diagnosis; Therapy; Prognosis; Child