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%. 1 Chronic 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][8] Here 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
| CASE PRESENTATIONAn 11-year-old previously healthy Hispanic female presented with waxing/waning malar rash exacerbated by sunlight (Figure 1), submandibular lymphadenopathy, scleritis/keratitis, and oral ulcers/abscesses. Examination revealed an enlarged submandibular lymph node, hemorrhagic crusted papules in sun-exposed areas; and conjunctival injection. She had no hepatosplenomegaly. Complete blood counts, electrolytes, creatinine, liver enzymes, and lactate dehydrogenase were normal. Antinuclear antibody was negative. EBV antibody panel was positive for