Abstract:
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 × 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.
journal_name
Indian J Dermatoljournal_title
Indian journal of dermatologyauthors
Vanikar AV,Trivedi HL,Patel RD,Kanodia KV,Modi PR,Shah VRdoi
10.4103/0019-5154.92667subject
Has Abstractpub_date
2012-01-01 00:00:00pages
9-11issue
1eissn
0019-5154issn
1998-3611pii
IJD-57-9journal_volume
57pub_type
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