A new therapy in Epstein-Barr virus-associated lymphoproliferative disease: a case report and a revision of the literature
A new therapy in Epstein-Barr virus-associated lymphoproliferative disease: a case report and a revision of the literature
Blog Article
Abstract Background Systemic chronic active Epstein-Barr virus infection is an extremely rare childhood disease.Since chronic active Epstein-Barr virus infection can trigger the onset of Epstein-Barr virus-associated lymphoproliferative disease.The clinical manifestations of the disease vary according to the site of involvement; therefore, management may be challenging.
Currently, there are no standardized guidelines for treating Chronic active Epstein-Barr virus infection effectively.Case presentation We report a case of chronic active Epstein-Barr virus water bottle infection in a 5-year-old Chinese boy with intestinal, vascular, and neurological involvement.At age of 2 years and 7 months old, he had hepatomegaly and been diagnosed with Epstein-Barr virus infection.
After treatment, he showed some clinical improvement.At age of 3 years and 3 months old, he presented with recurrent fever and diarrhea.Then he received methylprednisolone for 1 year and his symptoms ameliorated.
At the age of 5 years, his symptoms recurred and had gastrointestinal hemorrhage and developed polyuria, frequent convulsions and hyponatremia.He was transferred to our hospital for further management.He was unconscious on admission and was diagnosised Epstein-Barr virus-lymphoproliferative disorder, based on the results in situ hybridization of EBV-encoded miRNA in sigmoid colon.
Three-dimensional CT angiography demonstrated an aneurysm in the right internal carotid artery.Abdominal CT showed dilatation of vessels in part of the intestinal wall.He was also diagnosised Epstein-Barr Magnetic Blocks virus encephalitis based on the elevated Epstein-Barr virus antibody titers and presence of Epstein-Barr virus DNA in the Cerebrospinal Fluid.
A repeated duodenal artery embolization and symptomatic therapy could not control the hemorrhage after admission.He subsequently received treatment with ganciclovir, glucocorticoid, thalidomide, and propranolol.Hemorrhage was controlled in 5 days; his symptoms improved.
The fever did not recur and the CSF pressure was also normalized.A follow-up CT at 3 months after admission showed regression of the aneurysm in the right internal carotid artery and the vascular lesion in the duodenum.Discussion and conclusions A new treatment protocol including thalidomide and propranolol resulted in a marked improvement in his clinical symptoms, and shows promise as a novel and effective therapeutic approach for Chronic active Epstein-Barr virus infection-associated lymphoproliferative disorder.