Allogenic hematopoietic stem cell transplantation in pemphigus vulgaris: A single-center experience

• Main Authors: Aruna V Vanikar, Hargovind L Trivedi, Rashmi D Patel, Kamal V Kanodia, Pranjal R Modi, Veena R Shah
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2012-01-01
• Series: Indian Journal of Dermatology
• Subjects: Bullous lesions; hematopoietic stem cell transplantation; pemphigus vulgaris
• Online Access: http://www.e-ijd.org/article.asp?issn=0019-5154;year=2012;volume=57;issue=1;spage=9;epage=11;aulast=Vanikar
• ISSN: 0019-5154; 1998-3611

Background: Pemphigus vulgaris (PV), an autoimmune disorder characterized by blistering skin/mucus membrane lesions, is mediated by desmoglein-3 autoantibodies. We carried out a prospective clinical trial of hematopoietic stem cell transplantation (HSCT) in thymus, bone marrow (BM) and periphery to reconstitute central and peripheral arms of self-tolerance. Materials and Methods: Eleven (M:F=5:6) patients with mean age 33.5 years and mean duration of disease 22.8 months, having painful pruritic blisters and ulcers resistant to corticosteroids, were treated with cytokine-stimulated allogeneic HSCT (mean dose: 21.8 Χ 10 8 cells/kg BW) from blood group-matched related donors. BM with mean CD34+ count 1.1% was inoculated into thymus, marrow and periphery, followed by two peripheral blood stem cell (PBSC) infusions. Results: Recovery began within 24 hours of HSCT and new lesions stopped after 6 months. No graft versus host disease (GvHD)/adverse effect was observed in any patient/donor. Over a mean follow-up of 8.02 years, all patients were well without recurrence/new lesions. Conclusion: Drug-resistant PV can be successfully and safely treated by allogeneic HSCT.