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Published on: December 8, 2023
Allogenic hematopoietic stem cell transplantation in pemphigus vulgaris: a single-center experience
Aruna V Vanikar1, Hargovind L Trivedi, Rashmi D Patel
1Department of Pathology, Laboratory Medicine, Transfusion Services and Immunohematology, G. R. Doshi and K. M. Mehta Institute of Kidney Diseases and Research Centre (IKDRC) and Dr. H. L. Trivedi Institute of Transplantation Sciences (ITS), Ahmedabad, India. vanikararuna@yahoo.com
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.
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