CAJ | 학술논문

目的探讨儿童难治性自身免疫性疾病进行自体外周血干细胞动员采集的安全性和CD34+细胞分选纯化的可行性及其临床意义.方法 8例儿童难治性自身免疫性疾病,包括4例系统性红斑狼疮、2例皮肌炎,1例幼年型类风湿关节炎和1例多发性硬化,予行CD34+细胞纯化的自体外周血干细胞移植.首先采用环磷酰胺(CTX)联合粒细胞集落刺激因子(G-CSF)方案动员外周血干细胞,然后采用CS-3000血细胞分离机采集外周血,通过CliniMACS细胞分选仪分选自体外周血CD34+细胞,将其用保养液配置冻存于-80℃冰箱.采用非清髓内去除T的预处理方案,即卡氮芥+足叶乙苷+阿糖胞苷+马法兰+抗胸腺球蛋白(ATG)或CTX+ATG或CTX+马法兰+ATG,于第0天回输自体外周血CD34+细胞.结果儿童能够耐受自体外周血干细胞动员采集过程,无动员相关死亡,动员后获得的单个核细胞数和CD34+细胞数的平均值分别为8.35×108/kg和7.92×106/kg,纯化后的白体外周血CD34+和CD3+细胞数的平均值分别为6.28×106/kg和0.71×105/kg.回输后中性粒细胞和血小板的植入中位时间分别为+11d和+15 d.结论经CTX联合G-CSF方案可动员出足量的外周血干细胞,经CS-3000血细胞分离机采集可获得足够的单个核细胞,在动员过程中原发病无明显进展恶化,患儿能耐受动员方案,采集过程顺利安全;经CliniMACS细胞分选仪分选的自体外周血CD34+细胞纯度高,移植后造血恢复;采用CD34+细胞纯化的自体外周血移植治疗是常规治疗无效的儿童难治性自身免疫性疾病的可选择治疗措施之一.
목적 탐토인동난치성자신면역성질병진행자체외주혈간세포동원채집적안전성화CD34+세포분선순화적가행성급기림상의의.방법 8례인동난치성자신면역성질병,포괄4례계통성홍반랑창、2례피기염,1례유년형류풍습관절염화1례다발성경화,여행CD34+세포순화적자체외주혈간세포이식.수선채용배린선알(CTX)연합립세포집락자격인자(G-CSF)방안동원외주혈간세포,연후채용CS-3000혈세포분리궤채집외주혈,통과CliniMACS세포분선의분선자체외주혈CD34+세포,장기용보양액배치동존우-80℃빙상.채용비청수내거제T적예처리방안,즉잡담개+족협을감+아당포감+마법란+항흉선구단백(ATG)혹CTX+ATG혹CTX+마법란+ATG,우제0천회수자체외주혈CD34+세포.결과 인동능구내수자체외주혈간세포동원채집과정,무동원상관사망,동원후획득적단개핵세포수화CD34+세포수적평균치분별위8.35×108/kg화7.92×106/kg,순화후적백체외주혈CD34+화CD3+세포수적평균치분별위6.28×106/kg화0.71×105/kg.회수후중성립세포화혈소판적식입중위시간분별위+11d화+15 d.결론 경CTX연합G-CSF방안가동원출족량적외주혈간세포,경CS-3000혈세포분리궤채집가획득족구적단개핵세포,재동원과정중원발병무명현진전악화,환인능내수동원방안,채집과정순리안전;경CliniMACS세포분선의분선적자체외주혈CD34+세포순도고,이식후조혈회복;채용CD34+세포순화적자체외주혈이식치료시상규치료무효적인동난치성자신면역성질병적가선택치료조시지일.
Objective To explore the safety of mobilization and collection as well as the feasibility of selection of autologous peripheral blood stem cells (auto-PBSC) from patients with juvenile severe autoimmune diseases (AID) for autologous hematopoietic stem cell transplantation (auto-HSCT). The clinical significance of these procedure is evaluated. Methods Eight patients with AID, including four patients with systemic lupus erythematosus(SLE),two patients with dermatomysoitis, one patient with juvenile rheumatoid arthritis (JRA), one patient with multiple sclerosis(MS),underwent auto-HSCT. Auto-PBSCs were mobilized in 8 patients using cyclophosphamide(CTX) and granulocyte colony-stimulating factor (G-CSF), and their PBSCs were collected by CS-3000 Blood Cell Separator, then the CD34+cells were selected and purified by CliniMACS CD34+cell selection device. The CD34+ cells were frozenand preserved under -80 ℃ ALL patients received non-myeloablative or lymphoablative conditioning regimens which consisted of CTX/Mel/ATG or CTX/ATG or BEAM/ATG. All patient received CD34+ cells transplantation. The safety of mobilization and collection process of auto-PBSC as well asthe feasibility of selection and purification of CD34+cells were recorded and hematopoietic reconstruction were evaluated. Results All patients tolerated the collection process well, and there was no mobilization-related mortality. The number of collected MNCs and CD34+ cells were 8.35×108/kg and 7.92×106/kg respectively. The number of CD34+ and CD3+ cells after purification was 6.28×106/kg and0.71 ×105/kg respectively. The mean granulocytes and platelet engraftment occurred on days 11 and 15 after G-CSF regimen, and they can be collected using CS-3000 instrument. PBSC mobilization and collection from patients with juvenile severe AID is safe. The CD34+ cell can be highly purified. The auto-PBSC CD34+cell transplantation is an alternative therapy for severe AIDs that do not respond to conventional treatments.