title: Induction of an inhibitor antibody to factor XI in a patient with severe inherited factor XI deficiency by Rh immune globulin.
abstract: In this paper, we report an inhibitor antibody to factor XI (FXI) in a woman with severe inherited FXI deficiency, induced by FXI present in an Rh immune globulin preparation. The patient is homozygous for the Glu117Stop mutation, associated with a FXI level of less than 1 U/dL. Unlike all previously described patients with severe FXI deficiency and an inhibitor, the patient had never been exposed to blood products. Following 3 injections of Rh immune globulin during pregnancy, she developed an inhibitor to FXI (8 Bethesda units) that was shown to bind specifically to FXI and inhibit factor IX cleavage by purified FXIa. The administered Rh immune globulin and 2 other similar products were shown to contain FXI. Clinicians should be aware of the potential for immunization of severely FXI-deficient patients by FXI present in Rh immune globulin preparations.
authors.affiliation: Amalia Biron Research Institute of Thrombosis and Hemostasis, Chaim Sheba Medical Center, Tel-Hashomer and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Michal
authors.initials: M
authors.lastname: Zucker
authors.forename: Ariella
authors.initials: A
authors.lastname: Zivelin
authors.forename: Jerome
authors.initials: J
authors.lastname: Teitel
authors.forename: Uri
authors.initials: U
authors.lastname: Seligsohn
mesh_terms: D000328:Adult; D004198:Disease Susceptibility / Q000276:immunology / Q000473:pathology; D005172:Factor XI / Q000037:antagonists & inhibitors* / Q000276:immunology*; D005173:Factor XI Deficiency / Q000276:immunology* / Q000473:pathology*; D005260:Female; D006801:Humans; D018029:Rho(D) Immune Globulin / Q000276:immunology*
chemical_list: D018029:Rho(D) Immune Globulin; D005172:Factor XI