Minegishi M, Ohashi Y, Kumaki S, Sasahara Y, Hayashi T, Asada H, Okuyama T, Hakozaki I, Sato T, Tsuchiya S
Department of Pediatric Oncology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.
Bone Marrow Transplant. 2001 Apr;27(8):883-6. doi: 10.1038/sj.bmt.1702999.
We report a case of a 5-year-old girl with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) who underwent cord blood (CB) stem cell transplantation (CBSCT) from an unrelated donor. The patient presented with persistent high-grade fever and hepatosplenomegaly. Because the disease was refractory to immunochemotherapy according to the HLH94 protocol, she received 2.0 x 10(7) CB nucleated cells/kg body weight (BW) after conditioning with BU/CY/etoposide. No acute GVHD developed, using FK506 for prophylaxis. The neutrophil count reached >0.5 x 10(9)/l by day 21 and the platelet count reached >50 x 10(9)/l by day 84. The patient recovered well with sequelae of neurological deficits more than 10 months after receiving CBSCT, without showing evidence of HLH or chronic GVHD. Real-time PCR proved applicable for estimation of the EBV load in PBMC of the patient. We conclude that CBSCT may be indicated for some cases of refractory EBV-HLH, who have no HLA-matched siblings and are therefore dependent on unrelated marrow donors.
我们报告了一例5岁患爱泼斯坦-巴尔病毒相关噬血细胞性淋巴组织细胞增生症（EBV-HLH）的女孩，她接受了来自非亲属供者的脐血（CB）干细胞移植（CBSCT）。该患者表现为持续高热和肝脾肿大。由于根据HLH94方案进行的免疫化疗对该疾病无效，她在接受白消安/环磷酰胺/依托泊苷预处理后，接受了2.0×10⁷个CB有核细胞/千克体重（BW）的移植。使用FK506进行预防，未发生急性移植物抗宿主病（GVHD）。中性粒细胞计数在第21天达到>0.5×10⁹/升，血小板计数在第84天达到>50×10⁹/升。该患者在接受CBSCT后10多个月恢复良好，伴有神经功能缺损后遗症，未显示HLH或慢性GVHD的迹象。实时聚合酶链反应（PCR）证明适用于评估该患者外周血单个核细胞（PBMC）中的EBV载量。我们得出结论，对于一些难治性EBV-HLH病例，如果没有HLA匹配的同胞，因此依赖非亲属骨髓供者，CBSCT可能是一种选择。
