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  

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

作　　者：吴南海[1] 龚小军[1] 栾佐[1] 王凯[1] 唐湘凤[1] 

机构地区：[1]海军总医院儿科

出　　处：《中国实用儿科杂志》2012年第7期522-525,共4页Chinese Journal of Practical Pediatrics

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8d至术前5d;环磷酰胺(CTX)60mg/(kg·d),术前3d至术前2d;依托泊苷(VP16)30mg/kg,术前4d。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16d重建粒系造血,术后37d重建巨核系造血,术后60d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62d出现皮肤Ⅱ度GVHD。移植预处理前EBV-DNA1.2×105拷贝/mL,术后14dEBV-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。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, diag- nosed 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＋VP,6 （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 6.34×10^8/kg nucleated cells and 3.80×10^6/kg CDE positive cells. GVHD prophylaxis con- sisted of cyclosporine A （CsA） and a short course of methotrexate （sMTX）. Results The hematologic recovery was fa- vorable. The patient achieved neutrophil engraftment at day ＋ 16;platelet engraftment was achieved at day ＋37 ; his hemo- gram completely recqvered 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 devel- oped cutaneous Grade II acute graft-versus-host disease （GVHD） at day ＋62. The EBV-DNA was 1.2×10^5 copies/ml before transplant, and changed-over to negative at day ＋ 14; after this, the EBV-DNA was monitored weekly, persistent- ly 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, hepatomega-ly, or splenomegaly; the previous lymphadenectasis at porta hepatic and aside abdominal aorta disap- peared. Computed tomography scan at thorax dis-played no lymphadenectasis at portopulmonary, the mediastinum was normal. Up to now, and the patient has been fol- lowed

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 

分 类 号：R72[医药卫生—儿科]