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Acquired factor XI deficiency and therapeutic plasma exchange
Geoffrey D Wool1, Angela Treml1, Jonathan L Miller1
1Department of Pathology, University of Chicago, Chicago, Illinois.
Journal of Clinical Apheresis
|October 14, 2017
Summary
Therapeutic plasma exchange (TPE) effectively managed acquired factor XI (FXI) deficiency in a pregnant lupus patient. TPE rapidly increased FXI levels and removed inhibitors, ensuring safe delivery with controlled maternal bleeding.
Area of Science:
- Hematology
- Obstetrics
- Immunology
Background:
- Congenital factor XI (FXI) deficiency presents with variable bleeding.
- Therapeutic plasma exchange (TPE) is used to increase FXI levels before procedures in congenital FXI deficiency.
- Acquired FXI deficiency can occur, particularly in autoimmune conditions.
Observation:
- A pregnant woman with lupus developed acquired FXI deficiency due to an IgG inhibitor, posing risks to both mother and fetus.
- Immune suppression failed to fully restore FXI levels.
- A multidisciplinary team utilized TPE with 100% plasma replacement during labor.
Findings:
- TPE successfully and isovolumetrically increased the mother's FXI levels and removed circulating inhibitors.
- The mother experienced controllable bleeding during a cesarean section post-TPE.
- The neonate exhibited no bleeding complications and normal FXI levels.
Implications:
- TPE is a viable strategy for managing acquired FXI deficiency, especially in pregnancy.
- This approach can acutely raise factor levels and clear inhibitors in acquired hemophilias.
- TPE offers a safe method for managing bleeding risks in pregnant patients with acquired coagulation factor deficiencies.