Introduction
Epstein-Barr Virus (EBV) is a common human herpesvirus with a typically
benign clinical course, most commonly infecting children with an
estimated worldwide prevalence of more than 90%.1Chronic active Epstein-Barr virus (CAEBV) is a rare T- and NK-cell
lymphoproliferative disorder of EBV origin affecting children mostly in
Asia and the Americas.2 CAEBV can manifest in a broad
spectrum of diseases including a cutaneous disorder known as hydroa
vacciniforme-like lymphoproliferative disorder (HV-LPD) and a systemic
form with persistent or recurrent infectious mononucleosis-like symptoms
following primary EBV infection.3 HV-LPD presents with
vesicular lesions induced by sun-exposure. Systemic CAEBV can be
associated with a variety of symptoms including viral hepatitis,
hemophagocytic syndrome, coronary artery aneurysms (CAA), basal ganglia
calcification, oral ulcers, lymphoma, interstitial pneumonia, central
nervous system (CNS) disease, pulmonary arterial hypertension (PAH),
enteritis, and gastrointestinal perforations.4,5,6,7,8Here we present a case of a pediatric patient diagnosed with T-cell
associated CAEBV and HV-LPD treated with reduced intensity conditioning
(RIC) hematopoietic stem cell transplantation (HSCT) and complicated by
transient delirium, CAA, PAH, and fatal ruptured cerebral artery
aneurysm. This case was previously reported in two case
series.9,10