Catastrophic relapse of Evans syndrome five years after allogeneic BMT notwithstanding full donor chimerism. Terminal hemolytic-uremic syndrome.

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

Autoimmunity

journal_title

Autoimmunity

authors

Marmont AM,Gualandi F,Occhini D,Morandi F,Ferretti E,Pezzolo A,Strada P,Ravetti JL,Pistoia V,Falanga A,Bacigalupo A

doi

10.1080/08916930600847598

subject

Has Abstract

pub_date

2006-09-01 00:00:00

pages

505-11

issue

6

eissn

0891-6934

issn

1607-842X

pii

Q2KW387807863634

journal_volume

39

pub_type

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