ABSTRACT
Systemic sclerosis (SSc) is an uncommon connective tissue disease characterized by excessive collagen deposition within the skin and internal organs. Most patients with diffuse severe SSc are treated with immunosuppressive agents, but patients with advanced disease have very high 5-year mortality rates despite adequate therapy. We describe a patient with both diffuse cutaneous SSc and systemic lupus erythematosus who showed mixed chimerism 29 months after undergoing nonmyeloablative stem cell transplant. She experienced remission of both diseases.
Subject(s)
Immunosuppressive Agents/administration & dosage , Lupus Erythematosus, Systemic/therapy , Scleroderma, Systemic/therapy , Stem Cell Transplantation/methods , Transplantation Conditioning , Adult , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Risk Assessment , Scleroderma, Systemic/complications , Scleroderma, Systemic/diagnosis , Severity of Illness Index , Treatment OutcomeABSTRACT
The small number of progenitor cells is the major limitation to the use of umbilical cord blood (UCB) for the transplantation of adults. We tested the hypothesis that two units transplanted simultaneously could each contribute to haematopoietic reconstitution. A patient with advanced acute lymphocytic leukaemia received a mismatched, unrelated UCB transplant using units from two donors after conditioning. The recipient achieved a complete remission without graft-versus-host disease. Double chimaerism was documented in several leucocyte subpopulations; both units contributed to haematopoiesis until relapse. Triple chimaerism was present from relapse until death due to leukaemia. This approach may potentially improve UCB transplantation outcome for adults lacking a histocompatible donor.
