Division of Infectious Disease, Immunology, and Allergy, Saitama Children's Medical Center, Saitama, Japan.
Pediatr Blood Cancer. 2011 Oct;57(4):677-80. doi: 10.1002/pbc.23231. Epub 2011 Jun 16.
A 19-month-old male with Chediak-Higashi syndrome developed Epstein-Barr virus (EBV)-associated accelerated phase. Real-time polymerase chain reaction showed high EBV-DNA levels in plasma and peripheral blood mononuclear cells. His condition was refractory to conventional treatments for hemophagocytic lymphohistiocytosis, including corticosteroids, cyclosporine, and etoposide. In situ hybridization revealed higher proportion of EBER-1-positive cells in CD19+ cell fraction than in CD8+ cell fraction. Complete remission was achieved by combination therapy with rituximab and cyclosporine; subsequent bone marrow transplantation was successful. Combination therapy with rituximab and cyclosporine could be effective in patients with EBV-infected T and B cells.
一名 19 个月大的男性患有 Chediak-Higashi 综合征，发展为 EBV 相关的加速期。实时聚合酶链反应显示血浆和外周血单个核细胞中 EBV-DNA 水平较高。他的病情对噬血细胞性淋巴组织细胞增生症的常规治疗（包括皮质类固醇、环孢素和依托泊苷）无反应。原位杂交显示，在 CD19+细胞群中 EBER-1 阳性细胞的比例高于 CD8+细胞群。利妥昔单抗和环孢素联合治疗达到完全缓解；随后骨髓移植成功。利妥昔单抗和环孢素联合治疗可能对 EBV 感染的 T 和 B 细胞患者有效。
