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 |
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creator | Wool, Geoffrey D. 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. |
doi_str_mv | 10.1002/jca.21593 |
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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.</description><identifier>ISSN: 0733-2459</identifier><identifier>EISSN: 1098-1101</identifier><identifier>DOI: 10.1002/jca.21593</identifier><identifier>PMID: 29027258</identifier><language>eng</language><publisher>United States: Wiley Subscription Services, Inc</publisher><subject>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</subject><ispartof>Journal of clinical apheresis, 2018-06, Vol.33 (3), p.427-430</ispartof><rights>2017 Wiley Periodicals, Inc.</rights><rights>2018 Wiley Periodicals, Inc.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3203-4b7974bda7ead0ebe0f3cf133dbabb73cf50b531608304d9c23e06171fa319723</citedby><cites>FETCH-LOGICAL-c3203-4b7974bda7ead0ebe0f3cf133dbabb73cf50b531608304d9c23e06171fa319723</cites><orcidid>0000-0002-3335-2905</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fjca.21593$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fjca.21593$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,27924,27925,45574,45575</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29027258$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Wool, Geoffrey D.</creatorcontrib><creatorcontrib>Treml, Angela</creatorcontrib><creatorcontrib>Miller, Jonathan L.</creatorcontrib><title>Acquired factor XI deficiency and therapeutic plasma exchange</title><title>Journal of clinical apheresis</title><addtitle>J Clin Apher</addtitle><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.</description><subject>acquired inhibitor</subject><subject>Apheresis</subject><subject>Cesarean Section</subject><subject>Factor XI</subject><subject>Factor XI Deficiency - immunology</subject><subject>Factor XI Deficiency - therapy</subject><subject>Female</subject><subject>Humans</subject><subject>Immunoglobulin G</subject><subject>Lupus Erythematosus, Systemic - complications</subject><subject>Plasma</subject><subject>Plasma Exchange - methods</subject><subject>Pregnancy</subject><subject>therapeutic plasma exchange</subject><subject>Treatment Outcome</subject><issn>0733-2459</issn><issn>1098-1101</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kDtPwzAURi0EoqUw8AdQJCaGtNd2XMcDQ1XxKKrEAhKb5ccNTdUmqZMI-u8JpLAx3TscnU86hFxSGFMANlk7M2ZUKH5EhhRUGlMK9JgMQXIes0SoATmr6zUAKMXFKRkwBUwykQ7J7czt2jygjzLjmjJEb4vIY5a7HAu3j0zho2aFwVTYNrmLqo2ptybCT7cyxTuek5PMbGq8ONwReb2_e5k_xsvnh8V8towdZ8DjxEolE-uNROMBLULGXUY599ZYK7tfgBWcTiHlkHjlGEeYUkkzw6mSjI_Ide-tQrlrsW70umxD0U1qBqlMhJBMddRNT7lQ1nXATFch35qw1xT0dyjdhdI_oTr26mBs7Rb9H_lbpgMmPfCRb3D_v0k_zWe98gtNJnCX</recordid><startdate>201806</startdate><enddate>201806</enddate><creator>Wool, Geoffrey D.</creator><creator>Treml, Angela</creator><creator>Miller, Jonathan L.</creator><general>Wiley Subscription Services, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>K9.</scope><orcidid>https://orcid.org/0000-0002-3335-2905</orcidid></search><sort><creationdate>201806</creationdate><title>Acquired factor XI deficiency and therapeutic plasma exchange</title><author>Wool, Geoffrey D. ; Treml, Angela ; Miller, Jonathan L.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3203-4b7974bda7ead0ebe0f3cf133dbabb73cf50b531608304d9c23e06171fa319723</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>acquired inhibitor</topic><topic>Apheresis</topic><topic>Cesarean Section</topic><topic>Factor XI</topic><topic>Factor XI Deficiency - immunology</topic><topic>Factor XI Deficiency - therapy</topic><topic>Female</topic><topic>Humans</topic><topic>Immunoglobulin G</topic><topic>Lupus Erythematosus, Systemic - complications</topic><topic>Plasma</topic><topic>Plasma Exchange - methods</topic><topic>Pregnancy</topic><topic>therapeutic plasma exchange</topic><topic>Treatment Outcome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Wool, Geoffrey D.</creatorcontrib><creatorcontrib>Treml, Angela</creatorcontrib><creatorcontrib>Miller, Jonathan L.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><jtitle>Journal of clinical apheresis</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Wool, Geoffrey D.</au><au>Treml, Angela</au><au>Miller, Jonathan L.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Acquired factor XI deficiency and therapeutic plasma exchange</atitle><jtitle>Journal of clinical apheresis</jtitle><addtitle>J Clin Apher</addtitle><date>2018-06</date><risdate>2018</risdate><volume>33</volume><issue>3</issue><spage>427</spage><epage>430</epage><pages>427-430</pages><issn>0733-2459</issn><eissn>1098-1101</eissn><abstract>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.</abstract><cop>United States</cop><pub>Wiley Subscription Services, Inc</pub><pmid>29027258</pmid><doi>10.1002/jca.21593</doi><tpages>4</tpages><orcidid>https://orcid.org/0000-0002-3335-2905</orcidid></addata></record> |
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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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