title: 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.
authors.affiliation: Division of Hematology and Stem Cell Transplantation Center, Istituto G. Gaslini, Genova, Italy. alberto.marmont@hsanmartino.it
authors.forename: A M
authors.initials: AM
authors.lastname: Marmont
authors.forename: F
authors.initials: F
authors.lastname: Gualandi
authors.forename: D
authors.initials: D
authors.lastname: Occhini
authors.forename: F
authors.initials: F
authors.lastname: Morandi
authors.forename: E
authors.initials: E
authors.lastname: Ferretti
authors.forename: A
authors.initials: A
authors.lastname: Pezzolo
authors.forename: P
authors.initials: P
authors.lastname: Strada
authors.forename: J L
authors.initials: JL
authors.lastname: Ravetti
authors.forename: V
authors.initials: V
authors.lastname: Pistoia
authors.forename: A
authors.initials: A
authors.lastname: Falanga
authors.forename: A
authors.initials: A
authors.lastname: Bacigalupo
mesh_terms: D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune / Q000628:therapy*; D001323:Autoantibodies / Q000097:blood*; D016026:Bone Marrow Transplantation; D017809:Fatal Outcome; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology*; D006801:Humans; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000628:therapy*; D012008:Recurrence; D013577:Syndrome; D018183:Transplantation Chimera; D014184:Transplantation, Homologous
chemical_list: D001323:Autoantibodies