Abstract:

【Abstract】 Objective To explore the clinical efficacy and safety of autologous peripheral blood stem cell transplantation （APBSCT） in the treatment of pemphigus. Methods Totally, 3 patients with pemphigus vulgaris who responsed poorly to 6-month treatment with glucocorticoids or immunosuppressants or experienced aggrevation of disease and developed treatment-related complications, received APBSCT and were followed up for more than 5 years. There were 1 male and 2 females with an average age of 27.3 （21－39） years. The mobilization program included cyclophosphamide （CTX） 4 g/m2, recombinant human granulocyte colonystimulating factors（G-CSF） and Rituximab 375 mg/m2, and the preconditioning regimen included intravenous CTX （50 mg/kg per day on days －6, －5, －4, －3）, antithymocyte globulin at 2.5 mg/kg per day （on days －3, －2, －1 and 0） and Rituximab （600 mg/d on days 0 and 7）. Results All the 3 patients were successfully engrafted. The mean time for peripheral reconstruction: white blood cells 13.3 days （from day 11 to 16）, platelet 16.3 days （from day 16 to 17）. Monitoring of immunity indices and related antibodies showed no abnormality and the immune system was well reconstructed. No serious complications occurred during the follow up, and the patients′ quality of life was obviously improved. Conclusion APBSCT may be an effective and safe option for the treatment of pemphigus.

Key words: pemphigus