Treatment of severe primary Sjgren syndrome with peripheral blood stem cell transplantation
- VernacularTitle:大剂量化疗并自体外周血干细胞移植治疗重症原发性干燥综合征
- Author:
Xiao-Mei LENG
;
Yan ZHAO
;
Dao-Bin ZHOU
;
Ti SHEN
;
Tai-Sheng LI
;
Feng-Chun ZHANG
;
Xiao-Feng ZENG
;
Yi DONG
;
- Publication Type:Journal Article
- Keywords:
Sjgren syndrome;
Peripheral blood stem cell transplantation;
Therapy
- From:
Chinese Journal of Rheumatology
2001;0(04):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the feasibility,efficacy and safety of high dose chemotherapy (HDC)and autologous peripheral blood stem cell transplantation(PBSCT)with CD34~+ cell selection in patients with severe primary Sjgren's syndrome(pSS).Methods Three patients with persistent and severe pSS de- spite of glucocorticoid and immunosuppressive treatment were enrolled from 1999.All patients underwent high dose chemotherapy and peripheral blood stem cell infusion with CD34~+ cell selection.Autologous hematopoietic stem cells were mobilized with 2～3 g/m~2 CTX(total dosage,infused in two days)and 5?g/kg granulocyte colony stimulating factor(G-CSF),enriched with CD34~+ cell selection by CliniMACS,and reinfused after con- ditioning with 200 mg/kg CTX and 90 mg/kg swine antithymocyte globulin or 200 mg/kg CTX and total body irradiation of 4 Gy.Results One patient got complete remission after 2 times of mobilization,so no condi- tioning and transplantation were given.Other 2 patients completed the mobilization and leukapheresis proce dures successfully,and proceeded to receive conditioning and transplantation.All patients had rapid hematopoi- etic reconstitution.Three patients were followed up for 48 months,60 months and 18 months,respectively.All patients were free from abnormal activity of B lymphocytes.The titer of antibody decreased and anti-SSB anti- bodies of 2 patients turned to negative.Aggregation of focal lymphocytes in labial gland disappeared in 1 pa- tient after PBSCT.Improvement of pulmonary dysfunction and reversibility of interstitial pulmonary fibrosis fol- lowing hematopoietic stem cell transplantation were observed in 2 patients.Conclusion The abnormal activity of B lymphocytes in pSS patients can be controlled successfully with PBSCT.High close chemotherapy followed by peripheral blood stem cell transplantation with CD34~+ cell selection is feasible and safe.
