[Application of CD34+ autologous peripheral progenitor cell transplant in the treatment of children with refractory SLE].
OBJECTIVE
Systematic lupus erythematosis (SLE) is a severe disease which affects the patient for many years and there is no radical cure for the disease. To explore a possible way to treat children with refractory SLE, the authors treated 2 children with grade III and IV lupus nephropathy for 5 years and 7 years respectively, mainly presented with persistent thrombocytopenia, proteinuria, pleural effusion with CD34(+) autologous peripheral progenitor cells transplantation.


METHODS
Mobilized with G-CSF and collected with CS-3000 Cell Separator, passed through the CliniMacs CD34(+) cell selection device, the count of CD34(+) cells obtained reached 1.0 x 10(6)/kg and 1.7 x 10(6)/kg, respectively with the remaining of 2.0 x 10(5)/kg and 1.0 x 10(4)/kg of CD3(+) cells individually. The selected CD34(+) cells were frozen at -80 degrees C. The conditioning regimen consisted of cyclophosphamide [50 mg/(kg x day) for 4 days] plus ATG [Fresennius S 5 mg/(kg x day) for 3 days]. After 48 h treatment with cyclophosphamide, the frozen stem cells were infused back to the patients.


RESULTS
Neutrophils recovered on 9 and 7 days after transplantation respectively in these 2 cases. Beginning from 15 days, the platelet count recovered and remained at over 100 x 10(9)/L. The sign of Cushing's syndrome disappeared completely 3 months after transplantation because discontinuing the steroid. One child's height had a 5 cm increase within 6 months after stopping steroid and this was the first height gain during the 7 years since she had had the disease. Till this paper was written, these 2 children were followed up for 13 months and 6 months, respectively, all the original symptoms and autoantibodies related to autoimmune disorders disappeared. But the cell-mediated immunity did not recover yet with the CD4(+) cell level still remained at a lower level.


CONCLUSIONS
The effect of CD34(+) autologous peripheral progenitor cell transplantation on the children with refractory SLE was satisfactory so far, but the long-term effect remains to be confirmed by further studies on more cases.
Chen
Jing
J
Department of Hematology/Oncology, Xinhua Hospital, Shanghai Children's Medical Center, Shanghai Second Medical University, Shanghai 200127, China.
Gu
Long-jun
LJ
Zhao
Hui-jun
HJ
Xue
Hui-liang
HL
Zheng
Yi
Y
Xie
Xiao-juan
XJ
Chen
Jing
J
Li
Li
L
Xu
Chong
C
Wang
Yao-ping
YP
D000293:Adolescent; D018952:Antigens, CD34 / Q000276:immunology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000276:immunology / Q000628:therapy*; D036102:Peripheral Blood Stem Cell Transplantation; D014182:Transplantation, Autologous; D016896:Treatment Outcome
D018952:Antigens, CD34