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Autologous peripheral blood stem and progenitor (CD34+) cell transplantation for systemic lupus erythematosus complicated by Evans Syndrome
Author(s): Musso M, Porretto F, Crescimanno A, Bondi F, Polizzi V, Scalone R, Mariani G
Source: LUPUS    Volume: 7    Issue: 7    Pages: 492-494    Published: 1998  
Times Cited: 49     References: 10     
Abstract: Immunoablation followed by allogeneic stem cell (SC) transplantation has been shown to be capable of curing a large spectrum of experimental autoimmune disorders. hereditary and/or induced. Superimposable results, albeit with some exceptions, have been obtained in human patients affected by coincidental autoimmune and blood diseases. However, both because of encouraging experimental results and of the procedure's greater safety, autologous SC are being increasingly utilized worldwide. Case reports are being collected in the registry of the European Group for Blood and Marrow Transplantation (EBMT)/European League against Rheumatism (EULAR) Autoimmune Disease Stem Cell Project.

Among the severe autoimmune diseases (SADs), which are the target of autologous transplantation. severe refractory systemic lupus erythematosus (SLE) is a condition which may benefit from this procrdure. We report here the case of a 19 year old female patient with a six year history of SLE with secondary antiphospholipid syndrome (APS), who later developed refractory Evans syndrome. She was transplanted with autologous mobilized CD34+ SC and progenitor cells after conditioning with cyclosphosphamide, anti-T lymphocyte globulin and prednisone. Eight months after transplant, the patient is alive and well, with normal blood counts and persistent low-titre direct antiglobulin (DAT,, Coombs) and anti-nuclear antibody (ANA) tests. Anti-double stranded DNA antibody (Anti-dsDNA), lupus anticoagulant tests and anti-cardiolipin antibody (ACA) test are negative.

Document Type: Article
Language: English
Reprint Address: Musso, M (reprint author), Univ Palermo, Div Ematol, TMO, Via Vespro 129, I-90127 Palermo, Italy
Addresses:
1. Univ Palermo, Div Ematol, TMO, I-90127 Palermo, Italy
2. Univ Palermo, Bone Marrow Unit, I-90127 Palermo, Italy
Publisher: STOCKTON PRESS, HOUNDMILLS, BASINGSTOKE RG21 6XS, HAMPSHIRE, ENGLAND
Subject Category: Rheumatology
IDS Number: 127FK
ISSN: 0961-2033
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