Application of CD34+ autologous peripheral progenitor cell transplant in the treatment of children with refractory SLE.
- Author:
Jing CHEN
1
;
Long-jun GU
;
Hui-jun ZHAO
;
Hui-liang XUE
;
Yi ZHENG
;
Xiao-juan XIE
;
Jing CHEN
;
Li LI
;
Chong XU
;
Yao-ping WANG
Author Information
- Publication Type:Case Reports
- MeSH: Adolescent; Antigens, CD34; immunology; Female; Follow-Up Studies; Humans; Lupus Erythematosus, Systemic; immunology; therapy; Peripheral Blood Stem Cell Transplantation; Transplantation, Autologous; Treatment Outcome
- From: Chinese Journal of Pediatrics 2003;41(6):426-429
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVESystematic 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.
METHODSMobilized 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.
RESULTSNeutrophils 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.
CONCLUSIONThe 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.