Abstract:
:A patient with severe Evans syndrome received an allo-BMT from his HLA-identical sister on November, 2000. Full marrow and blood donor chimerism were achieved only after 5 donor lymphocyte infusions (DLI), and coincided with complete clinical remission and disappearence of auto-antibodies. Five years later, hemolytic anemia recurred with rapid increase of serum bilirubin to over 50 mg%: he responded to combined therapy, but died on day +17 from admission of an acute hemolytic uremic syndrome (HUS). All circulating blood cells, including erythrocytes, were 100% donor. Ex vivo cultured and expanded T and B cells from the peripheral blood were also 100% donor. The supernatants from B cell cultures, containing either IgM or IgG, did not react with a panel of erythrocytes. Thus in this typical autoimmune disease with a predominant B cell pathogenesis the donor immune system resulted "innocent of autoimmunity". The persistence of long-lived recipient autoreactive plasma-cell lines in survival niches, still producing autoantibodies, may be hypothesized for this and similar cases. The postulated graft-versus-autoimmunity (GVA) effect was apparently not sufficient to eradicate autoimmunity in this patient.
journal_name
Autoimmunityjournal_title
Autoimmunityauthors
Marmont AM,Gualandi F,Occhini D,Morandi F,Ferretti E,Pezzolo A,Strada P,Ravetti JL,Pistoia V,Falanga A,Bacigalupo Adoi
10.1080/08916930600847598subject
Has Abstractpub_date
2006-09-01 00:00:00pages
505-11issue
6eissn
0891-6934issn
1607-842Xpii
Q2KW387807863634journal_volume
39pub_type
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