中国实用儿科杂志

中国实用儿科杂志 ›› 2012, Vol. 27 ›› Issue (07): 522-525.

• 论著 • 上一篇    下一篇

造血干细胞移植治疗儿童EB病毒相关T/NK细胞
淋巴组织增殖性疾病疗效分析

吴南海，龚小军，栾    佐，王    凯，唐湘凤   

  1. 海军总医院儿科，北京    100048
  • 出版日期:2012-07-06 发布日期:2012-07-18

Treatment for a child with EBV-associated T/natural  killer-cell lymphoproliferative disorder by hematopoietic stem cell transplantation.    

WU Nan-hai，GONG Xiao-jun，LUAN Zuo，WANG Kai，TANG Xiang-feng. Department of pediatrics，Navy General Hospital of PLA，Beijing 100048，China   

  • Online:2012-07-06 Published:2012-07-18

摘要：

目的    探讨造血干细胞移植治疗儿童EB病毒（EBV）相关T/NK细胞淋巴组织增殖性疾病（EBV-T/NK-LPD）的效果。方法    海军总医院儿科2009-08-03收治1例EBV-T/NK-LPD患儿，年龄13岁6个月，经抗病毒药物、EB病毒特异性淋巴毒细胞（EBV-CTL）、白细胞介素-2（IL-2）及化疗治疗无效，行HLA 基因位点6/6全相合同胞外周血造血干细胞移植。预处理采用TBI/Cy＋VP16方案：全身放射治疗（TBI）总剂量12Gy，分8次，术前8 d至术前5 d；环磷酰胺（CTX）60 mg/（kg·d），术前3 d至术前2 d；依托泊苷（VP16） 30 mg/kg，术前4 d。输入干细胞数量：单个核细胞（MNC） 6.34 × 108 /kg，CD34+细胞 3.80 × 106/kg。移植物抗宿主病（GVHD）预防采用环孢菌素A＋短程甲氨蝶呤（CsA＋sMTX）。结果    移植后造血恢复顺利，术后16 d重建粒系造血，术后37 d重建巨核系造血，术后60 d后血象恢复正常。未出现严重感染、出血性膀胱炎、肝静脉闭塞症、间质性肺炎（IP）等并发症，未出现巨细胞病毒（CMV）感染。术后62 d 出现皮肤Ⅱ度GVHD。移植预处理前EBV-DNA 1.2 × 105拷贝/mL，术后14 d EBV-DNA转阴，造血重建后每周监测EBV-DNA持续阴性。移植后黄疸消失，肝功能恢复正常；复查颈部及腹部B超，未见颈部淋巴结肿大，肝脾未见异常，肝门及腹主动脉旁肿大淋巴结消失；肺CT扫描：未见肺门淋巴结肿大，纵隔无异常。移植后随访18个月，持续缓解。结论    国内首例造血干细胞移植治疗EBV-T/NK-LPD，植入顺利，能持续缓解病情并可能治愈该病。

关键词:  , EB病毒, T/NK细胞, 淋巴组织增殖性疾病, 造血干细胞移植, 儿童

Abstract:

Objective   To explore the therapeutic effect of hematopoietic stem cell transplantation for treatment of EBV-associated T/Natural Killer-cell Lymphoproliferative disorder in children. Methods   A 13.5-year old boy，diagnosed with EBV-associated T/natural killer-cell lymphoproliferative disorder （EBV-T/NK-LPD），received HLA-matched sibling PBSCT from his younger sister， as his disease was persistent after treatment with anti-virus drugs，EBV-CTL，interleukin-2 and chemotherapy. The conditioning regimen was TBI/CY+VP16  （total-body irradiation，12 Gy in 8 fractions，-8d～-5d；cyclophosphamide，60mg/kg×two doses，-3d～-2d；a single dose of etoposide，30mg/kg，-4d）. The infused PBSCs contained 6.34×108/kg nucleated cells and 3.80×106/ kg CD34 positive cells. GVHD prophylaxis consisted of cyclosporine A （CsA） and a short course of methotrexate （sMTX）.  Results   The hematologic recovery was favorable. The patient achieved neutrophil engraftment at day +16；platelet engraftment was achieved at day +37；his hemogram completely recovered at day +60.  The patient had no severe complications，such as fatal infections，hemorrhagic cystitis，veno-occlusive disease （VOD），interstitial pneumonia （IP），cytomegalovirus （CMV） infection，et al.  He developed cutaneous Grade II acute graft-versus-host disease （GVHD） at day +62. The EBV-DNA was 1.2×105 copies/ml before transplant，and changed-over to negative at day +14； after this，the EBV-DNA was monitored weekly，persistently negative. After transplantation，the patient’s jaundice disappeared，and the liver function normalized. Rechecking type-B ultrasonic on the cervix and the abdomen，there was no signs of lymphadenectasis at pars cervicalis，hepatomegaly，or splenomegaly；the previous lymphadenectasis at porta hepatic and aside abdominal aorta disappeared. Computed tomography scan at thorax displayed no lymphadenectasis at portopulmonary，the mediastinum was normal. Up to now，and the patient has been followed up for 18 months after transplantation，he is still in continuing complete remission. Conclusion   This was the first case who was treated by hematopoietic stem cell transplantation for  EBV-T/NK-LPD in Mainland China. The transplantation was successful. The patient with  EBV-T/NK-LPD can reach remission by hematopoietic stem cell transplantation，and likely to be cured with this therapy.

Key words:  , Epstein-Barr virus；T/natural killer-cell；lymphoproliferative disorder；hematopoietic stem cell transplantation；children