Chronic active Epstein-Barr virus infection (CAEBV) successfully treated with allogeneic peripheral blood stem cell...

Area of Science:

• Pediatric Hematology/Oncology
• Infectious Diseases
• Immunology

Background:

• Chronic Active Epstein-Barr Virus (CAEBV) infection can lead to severe complications, including T cell lymphoproliferative disorders.
• Accurate diagnosis relies on clinical presentation and molecular detection of the Epstein-Barr virus (EBV) genome.

Observation:

• A pediatric patient presented with clinical signs suggestive of CAEBV and T cell-based Hodgkin's-like disease.
• The Epstein-Barr virus (EBV) genome was detected in peripheral blood mononuclear cells (PBMC) and tumor cells, confirming the diagnosis.

Findings:

• Allogeneic peripheral blood stem cell transplantation (allo PBSCT) using an HLA-matched sibling donor was performed.
• Conditioning with busulfan (BU), etoposide (VP16), and cyclophosphamide (CY) was effective and well-tolerated.
• Complete eradication of the Epstein-Barr virus (EBV) was achieved within 3 months post-transplantation.
• The patient experienced chronic graft-versus-host disease (GVHD) of the liver but remained disease-free for 47 months post-transplantation.

Implications:

• Allogeneic peripheral blood stem cell transplantation (allo PBSCT) is a potent therapeutic strategy for eradicating the Epstein-Barr virus (EBV) genome in CAEBV.
• Allo PBSCT facilitates crucial immunological reconstitution, offering a potential cure for EBV-associated malignancies.
• This case highlights the efficacy of allo PBSCT in managing complex pediatric cases of CAEBV with associated T cell lymphoma.