Acquired factor XI deficiency and therapeutic plasma exchange

Congenital factor XI (FXI) deficiency is associated with a variable bleeding phenotype. Recent reports have documented the use of therapeutic plasma exchange to rapidly and isovolumetrically increase FXI levels before invasive procedures in patients with congenital FXI deficiency. We report a case o...

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Veröffentlicht in:Journal of clinical apheresis 2018-06, Vol.33 (3), p.427-430
Hauptverfasser: Wool, Geoffrey D., Treml, Angela, Miller, Jonathan L.
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Treml, Angela
Miller, Jonathan L.
description Congenital factor XI (FXI) deficiency is associated with a variable bleeding phenotype. Recent reports have documented the use of therapeutic plasma exchange to rapidly and isovolumetrically increase FXI levels before invasive procedures in patients with congenital FXI deficiency. We report a case of acquired FXI deficiency in a pregnant woman with lupus. We proved that the inhibitor was an IgG, therefore potentially capable of crossing the placenta. While immune suppression eliminated detectable circulating inhibitor, the woman's FXI remained quite low. A multi‐disciplinary team was formed and therapeutic plasma exchange with 100% plasma replacement was performed when the patient went into labor, to acutely raise her FXI level and remove any potential non‐neutralizing inhibitor. The mother had a controllable level of bleeding during post‐TPE cesarean section; the baby had no bleeding and the baby's FXI levels were not overtly abnormal. Therapeutic plasma exchange in acquired FXI deficiency (or other acquired hemophilias) can both acutely isovolumetrically raise factor levels and remove any circulating inhibitor.
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source Wiley-Blackwell Journals; MEDLINE
subjects acquired inhibitor
Apheresis
Cesarean Section
Factor XI
Factor XI Deficiency - immunology
Factor XI Deficiency - therapy
Female
Humans
Immunoglobulin G
Lupus Erythematosus, Systemic - complications
Plasma
Plasma Exchange - methods
Pregnancy
therapeutic plasma exchange
Treatment Outcome
title Acquired factor XI deficiency and therapeutic plasma exchange
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