1. Pediatric Infectious Diseases Unit, Pediatric Department, Hospital Dona Estefânia, CHULC, Lisboa, Portugal
2. Primary Immunodeficiencies Unit, Pediatric Department, Hospital Dona Estefânia, CHULC, Lisboa, Portugal
- Reunião internacional - 39th Annual Meeting of the European Society of Paediatric Infectious Diseases
Resumo:
Background: Epstein-Barr virus (EBV) infection is common in pediatric age, generally with a mild and self-limited presentation. However, it can lead to potentially life-threatening complications.
Case Presentation Summary: Four-year-old boy, with trisomy 21, surgically corrected congenital atrioventricular septal defect, presented with a 9-day history of fever, anorexia, hepatosplenomegaly, anasarca and epistaxis. The EBV viral load was positive (viral load of 4,8 log) in the presence of positive VCA IgG, negative VCA IgM and negative EBNA IgG. He fulfilled the hemophagocytic lymphohistiocytosis (HLH) criteria: hemoglobin 8.7 g/dL, platelets 39000/uL, hypofibrinogenemia (0.3 g/L) and hypertriglyceridemia (414 mg/dL), hyperferritinemia (3637,7 ng/mL), bone marrow hemophagocytosis and elevated sCD25 (46000 pg/mL). He also presented hypoalbuminemia, hyperbilirubinemia and a massive activation of T CD8+ cells (91.4%). Clinical improvement followed without any targeted therapeutic intervention. Interestingly, a monoclonal population of CD8+CD5- T cells was identified in the bone marrow and in the peripheral blood. An EBV-related T cell lymphoproliferative disease (LPD) related to an active EBV infection was assumed. The perforin, XIAP e SAP expression was normal, as was the degranulation and cytotoxicity. As the patient presented progressive clinical and analytical improvement, only careful monitorization was undertaken.
Learning Points/Discussion: EBV-related T-cell LPD are rare and, in many cases, fatal. We present a case of auto-limited T-cell LPD, probably related to the EBV-HLH immunodysregulation, A conservative wait and see approach was adopted and led to full resolution.
Palavras Chave: Epstein-Barr vírus; EBV-related T cell lymphoproliferative disease; T cell lymphoproliferative disease.