ArticleBlood cell therapy2025
Adult-Onset Epstein-Barr Virus-Associated T-Cell Lymphoproliferative Disease with Central Nervous System Involvement.
Article in Blood cell therapy, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Epstein-Barr virus (EBV)-associated T-cell lymphoproliferative disease (EBV+ T-cell LPD), including chronic active Epstein-Barr virus (CAEBV) disease, can affect the central nervous system (CNS) in children, albeit rarely reported in adults. We report a case of an adult with EBV+ T-cell LPD and CNS involvement treated with haploidentical stem cell transplantation (haplo-SCT) and post-transplant cyclophosphamide (PTCy). A 59-year-old woman developed a high fever and elevated lactate dehydrogenase (LDH) levels. Based on the clinical course, high serum EBV-DNA levels, and the presence of EBV-infected T cells in her bone marrow, she was diagnosed with EBV+ T-cell LPD. The patient initially responded to cooling therapy and chemotherapy, leading to a marked decrease in EBV-DNA levels. Subsequently, she experienced a relapse in the CNS, as evidenced by elevated EBV-DNA levels in the cerebrospinal fluid. After chemotherapy and whole-brain irradiation, the patient underwent haplo-SCT with PTCy, following reduced-intensity conditioning. The EBV-DNA load was undetectable 27 days after haplo-SCT, and MRI findings showed no apparent residual disease. This case highlights the importance of considering CNS relapse despite apparent virologic response in adult-onset EBV+ T-cell LPD. To our knowledge, this is among the few reported adult cases with clinically diagnosed CNS involvement successfully treated with allogeneic SCT.
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