Epstein-Barr Virus-Associated Lymphoproliferative Disorder in a Patient with Wiskott-Aldrich Syndrome after Bone Marrow Transplantation.
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A boy with Wiskott-Aldrich syndrome received T cell-depleted bone marrow transplantation (BMT) from his father. He developed Epstein-Barr virus (EBV) -associated lymphoproliferative disorder (LPD) after BMT. His primary symptoms were prolonged fever and systemic lymphadenopathy. During the course, M protein (IgG λ type) was observed, and it changed to IgM λ type after the therapy for LPD. We diagnosed EBV-associated LPD by axillary lymph node biopsy with EBV-encoded small RNA (EBER) in situ hybridization and polymerase chain reaction (PCR) of EBV-latent membrane protein-1 (LMP-1). We treated him with gancyclovir (DHPG), antilymphocyte globulin (ALG), antithymocyte globulin (ATG) and etoposide (VP-16). Each therapy was effective. Though he received second BMT from his HLA-matched sibling for reconstruction of his immune status, he died with uncontrollable sepsis 22 days after the second BMT. T cell-depleted BMT or the use of ATG increase the risk of EBV-associated LPD, because of suppressed cytotoxic T-lymphocyte function against latent EBV infection. As the therapy for EBV-associated LPD is very difficult, attention must be paid for earlier diagnosis and treatments, for example, reduction of immunosuppressive drugs or immunomodulatory drugs like cytokines, or chemotherapy if control of the disease is difficult.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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