A Case of Epstein-Barr Virus-Associated Peripheral T-Cell Lymphoma

• Published in: The Japanese Journal of Pediatric Hematology Vol. 11; no. 3; pp. 193 - 197
• Main Authors: NAYA, Mayumi, SAWADA, Tadashi, HIBI, Shigeyoshi, TABATA, Yasuhiro, KIHARA, Akio, IMASHUKU, Shinsaku, OSAWA, Minako, TODO, Shinjiro, NAKABAYASHI, Yoshinobu, HOJO, Makoto
• Format: Journal Article
• Language: Japanese
• Published: THE JAPANESE SOCIETY OF PEDIATRIC HEMATOLOGY/ONCOLOGY 1997; 特定非営利活動法人 日本小児血液・がん学会
• Subjects: bone marrow transplantation; Epstein-Barr virus; hemophagocytic lymphohistiocytosis; peripheral T-cell lymphoma
• ISSN: 0913-8706; 1884-4723
• DOI: 10.11412/jjph1987.11.193

A 4-year-old girl developed Epstein-Barr virus (EBV) -associated peripheral T-cell lymphoma (PTCL), showing hemophagocytic lymphohistiocytosis. Bone marrow smears showed an increase of atypical immuno-blasts with mature T-cell antigens and some histiocytic hemophagocytes. Southern blot analyses of bone marrow cells by TCR C-β-1 and EBV terminal probes revealed the monoclonal proliferation of EBV-infected T-cells. The patient received HLH-94 protocol and achieved remission in a month. At 50 days after the onset, however, the disease recurred. Combined chemotherapy with cyclosporin A improved her condition transiently. At the 7th month, she received an allogeneic bone marrow transplantation (BMT) from her HLA-identical brother. She is doing well 6 months after the transplantation. It is suggested that allogeneic BMT is an effective treatment for refractory EBV/PTCL.