Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation
A case of acquired hemophilia A (AHA) that developed in a patient with autoimmune pancreatitis (AIP) is presented. A 64-year-old woman was diagnosed with AIP in 2007. The symptoms resolved with prednisolone (PSL). Although the dose of PSL was tapered to 7.5 mg/day for maintenance, serum IgG4 levels...
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| Veröffentlicht in: | International journal of hematology 2018-09, Vol.108 (3), p.335-338 |
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| creator | Narazaki, Taisuke Haji, Shojiro Nakashima, Yasuhiro Tsukamoto, Yasuhiro Tsuda, Mariko Takamatsu, Akiko Ohno, Hirofumi Matsushima, Takamitsu Matsumoto, Tomoko Nogami, Keiji Shima, Midori Shiratsuchi, Motoaki Ogawa, Yoshihiro |
| description | A case of acquired hemophilia A (AHA) that developed in a patient with autoimmune pancreatitis (AIP) is presented. A 64-year-old woman was diagnosed with AIP in 2007. The symptoms resolved with prednisolone (PSL). Although the dose of PSL was tapered to 7.5 mg/day for maintenance, serum IgG4 levels remained high. She suddenly presented with subcutaneous bleeding in 2015. Her activated partial thromboplastin time was prolonged (80.0 s). A mixing test showed an inhibitor pattern, factor VIII (FVIII) activity was less than 1%, and FVIII inhibitor was 290 BU/mL. She was diagnosed with AHA. Her serum IgG4 was elevated to 133 mg/dL. She was treated first with PSL alone, but she developed bladder tamponade. Cyclophosphamide and activated prothrombin complex concentrate were combined with PSL. She then achieved hemostasis, and FVIII inhibitor disappeared. FVIII inhibitor had been detected since PSL was tapered and AHA recurred two months later. An enzyme-linked immunosorbent assay showed that the inhibitor was mainly IgG4 and IgG1. This case suggests that elevation of IgG4 may be associated with the development of both AHA and AIP. |
| doi_str_mv | 10.1007/s12185-018-2441-3 |
| format | Article |
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A 64-year-old woman was diagnosed with AIP in 2007. The symptoms resolved with prednisolone (PSL). Although the dose of PSL was tapered to 7.5 mg/day for maintenance, serum IgG4 levels remained high. She suddenly presented with subcutaneous bleeding in 2015. Her activated partial thromboplastin time was prolonged (80.0 s). A mixing test showed an inhibitor pattern, factor VIII (FVIII) activity was less than 1%, and FVIII inhibitor was 290 BU/mL. She was diagnosed with AHA. Her serum IgG4 was elevated to 133 mg/dL. She was treated first with PSL alone, but she developed bladder tamponade. Cyclophosphamide and activated prothrombin complex concentrate were combined with PSL. She then achieved hemostasis, and FVIII inhibitor disappeared. FVIII inhibitor had been detected since PSL was tapered and AHA recurred two months later. An enzyme-linked immunosorbent assay showed that the inhibitor was mainly IgG4 and IgG1. This case suggests that elevation of IgG4 may be associated with the development of both AHA and AIP.</description><identifier>ISSN: 0925-5710</identifier><identifier>EISSN: 1865-3774</identifier><identifier>DOI: 10.1007/s12185-018-2441-3</identifier><identifier>PMID: 29582334</identifier><language>eng</language><publisher>Tokyo: Springer Japan</publisher><subject>Bladder ; Bleeding ; Case Report ; Coagulation factors ; Cyclophosphamide ; Enzyme-linked immunosorbent assay ; Factor VIII deficiency ; Hematology ; Hemophilia ; Hemostasis ; Hemostatics ; Immunoglobulin G ; Inhibitors ; Medicine ; Medicine & Public Health ; Oncology ; Pancreatitis ; Prednisolone ; Prothrombin ; Tamponade ; Thromboplastin</subject><ispartof>International journal of hematology, 2018-09, Vol.108 (3), p.335-338</ispartof><rights>The Japanese Society of Hematology 2018</rights><rights>International Journal of Hematology is a copyright of Springer, (2018). All Rights Reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c396t-bb1b9703b72410cea44c17736adb92f3530f40266d818323fba0c6af3343bd033</citedby><cites>FETCH-LOGICAL-c396t-bb1b9703b72410cea44c17736adb92f3530f40266d818323fba0c6af3343bd033</cites><orcidid>0000-0002-0403-0122</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s12185-018-2441-3$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s12185-018-2441-3$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,780,784,27924,27925,41488,42557,51319</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/29582334$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Narazaki, Taisuke</creatorcontrib><creatorcontrib>Haji, Shojiro</creatorcontrib><creatorcontrib>Nakashima, Yasuhiro</creatorcontrib><creatorcontrib>Tsukamoto, Yasuhiro</creatorcontrib><creatorcontrib>Tsuda, Mariko</creatorcontrib><creatorcontrib>Takamatsu, Akiko</creatorcontrib><creatorcontrib>Ohno, Hirofumi</creatorcontrib><creatorcontrib>Matsushima, Takamitsu</creatorcontrib><creatorcontrib>Matsumoto, Tomoko</creatorcontrib><creatorcontrib>Nogami, Keiji</creatorcontrib><creatorcontrib>Shima, Midori</creatorcontrib><creatorcontrib>Shiratsuchi, Motoaki</creatorcontrib><creatorcontrib>Ogawa, Yoshihiro</creatorcontrib><title>Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation</title><title>International journal of hematology</title><addtitle>Int J Hematol</addtitle><addtitle>Int J Hematol</addtitle><description>A case of acquired hemophilia A (AHA) that developed in a patient with autoimmune pancreatitis (AIP) is presented. A 64-year-old woman was diagnosed with AIP in 2007. The symptoms resolved with prednisolone (PSL). Although the dose of PSL was tapered to 7.5 mg/day for maintenance, serum IgG4 levels remained high. She suddenly presented with subcutaneous bleeding in 2015. Her activated partial thromboplastin time was prolonged (80.0 s). A mixing test showed an inhibitor pattern, factor VIII (FVIII) activity was less than 1%, and FVIII inhibitor was 290 BU/mL. She was diagnosed with AHA. Her serum IgG4 was elevated to 133 mg/dL. She was treated first with PSL alone, but she developed bladder tamponade. Cyclophosphamide and activated prothrombin complex concentrate were combined with PSL. She then achieved hemostasis, and FVIII inhibitor disappeared. FVIII inhibitor had been detected since PSL was tapered and AHA recurred two months later. An enzyme-linked immunosorbent assay showed that the inhibitor was mainly IgG4 and IgG1. This case suggests that elevation of IgG4 may be associated with the development of both AHA and AIP.</description><subject>Bladder</subject><subject>Bleeding</subject><subject>Case Report</subject><subject>Coagulation factors</subject><subject>Cyclophosphamide</subject><subject>Enzyme-linked immunosorbent assay</subject><subject>Factor VIII deficiency</subject><subject>Hematology</subject><subject>Hemophilia</subject><subject>Hemostasis</subject><subject>Hemostatics</subject><subject>Immunoglobulin G</subject><subject>Inhibitors</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Oncology</subject><subject>Pancreatitis</subject><subject>Prednisolone</subject><subject>Prothrombin</subject><subject>Tamponade</subject><subject>Thromboplastin</subject><issn>0925-5710</issn><issn>1865-3774</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNp1kEtLxDAUhYMoOj5-gBspuHFTvTdJm3Q5iI6C4EZxGdI0ncnQx5i0iv_eDPUBgqsL93z33MMh5BThEgHEVUCKMksBZUo5x5TtkBnKPEuZEHyXzKCgWZoJhANyGMIaAAVwsU8OaJFJyhifkZe5eR2dt1Wysm2_WbnG6WSe6BB64_QQ9-9uWCV6HHrXtmNnk43ujLd6cIMLkxisH9vkfrngiW3sW5T67pjs1boJ9uRrHpHn25un67v04XFxfz1_SA0r8iEtSywLAawUlCMYqzk3KATLdVUWtGYZg5oDzfNKomSU1aUGk-s6ZmdlBYwdkYvJd-P719GGQbUuGNs0urP9GBQFLIBjluURPf-DrvvRdzHdlpKcScFkpHCijO9D8LZWG-9a7T8Ugtq2rqbWVTxR29bVNsTZl_NYtrb6ufiuOQJ0AkKUuqX1v6__d_0EAvqL5g</recordid><startdate>20180901</startdate><enddate>20180901</enddate><creator>Narazaki, Taisuke</creator><creator>Haji, Shojiro</creator><creator>Nakashima, Yasuhiro</creator><creator>Tsukamoto, Yasuhiro</creator><creator>Tsuda, Mariko</creator><creator>Takamatsu, Akiko</creator><creator>Ohno, Hirofumi</creator><creator>Matsushima, Takamitsu</creator><creator>Matsumoto, Tomoko</creator><creator>Nogami, Keiji</creator><creator>Shima, Midori</creator><creator>Shiratsuchi, Motoaki</creator><creator>Ogawa, Yoshihiro</creator><general>Springer Japan</general><general>Springer Nature B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7RV</scope><scope>7T5</scope><scope>7T7</scope><scope>7TM</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FD</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>C1K</scope><scope>CCPQU</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-0403-0122</orcidid></search><sort><creationdate>20180901</creationdate><title>Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation</title><author>Narazaki, Taisuke ; Haji, Shojiro ; Nakashima, Yasuhiro ; Tsukamoto, Yasuhiro ; Tsuda, Mariko ; Takamatsu, Akiko ; Ohno, Hirofumi ; Matsushima, Takamitsu ; Matsumoto, Tomoko ; Nogami, Keiji ; Shima, Midori ; Shiratsuchi, Motoaki ; Ogawa, Yoshihiro</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c396t-bb1b9703b72410cea44c17736adb92f3530f40266d818323fba0c6af3343bd033</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Bladder</topic><topic>Bleeding</topic><topic>Case Report</topic><topic>Coagulation factors</topic><topic>Cyclophosphamide</topic><topic>Enzyme-linked immunosorbent assay</topic><topic>Factor VIII deficiency</topic><topic>Hematology</topic><topic>Hemophilia</topic><topic>Hemostasis</topic><topic>Hemostatics</topic><topic>Immunoglobulin G</topic><topic>Inhibitors</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Oncology</topic><topic>Pancreatitis</topic><topic>Prednisolone</topic><topic>Prothrombin</topic><topic>Tamponade</topic><topic>Thromboplastin</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Narazaki, Taisuke</creatorcontrib><creatorcontrib>Haji, Shojiro</creatorcontrib><creatorcontrib>Nakashima, Yasuhiro</creatorcontrib><creatorcontrib>Tsukamoto, Yasuhiro</creatorcontrib><creatorcontrib>Tsuda, Mariko</creatorcontrib><creatorcontrib>Takamatsu, Akiko</creatorcontrib><creatorcontrib>Ohno, Hirofumi</creatorcontrib><creatorcontrib>Matsushima, Takamitsu</creatorcontrib><creatorcontrib>Matsumoto, Tomoko</creatorcontrib><creatorcontrib>Nogami, Keiji</creatorcontrib><creatorcontrib>Shima, Midori</creatorcontrib><creatorcontrib>Shiratsuchi, Motoaki</creatorcontrib><creatorcontrib>Ogawa, Yoshihiro</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Nursing & Allied Health Database</collection><collection>Immunology Abstracts</collection><collection>Industrial and Applied Microbiology Abstracts (Microbiology A)</collection><collection>Nucleic Acids Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Technology Research Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing & Allied Health Premium</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><jtitle>International journal of hematology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Narazaki, Taisuke</au><au>Haji, Shojiro</au><au>Nakashima, Yasuhiro</au><au>Tsukamoto, Yasuhiro</au><au>Tsuda, Mariko</au><au>Takamatsu, Akiko</au><au>Ohno, Hirofumi</au><au>Matsushima, Takamitsu</au><au>Matsumoto, Tomoko</au><au>Nogami, Keiji</au><au>Shima, Midori</au><au>Shiratsuchi, Motoaki</au><au>Ogawa, Yoshihiro</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation</atitle><jtitle>International journal of hematology</jtitle><stitle>Int J Hematol</stitle><addtitle>Int J Hematol</addtitle><date>2018-09-01</date><risdate>2018</risdate><volume>108</volume><issue>3</issue><spage>335</spage><epage>338</epage><pages>335-338</pages><issn>0925-5710</issn><eissn>1865-3774</eissn><abstract>A case of acquired hemophilia A (AHA) that developed in a patient with autoimmune pancreatitis (AIP) is presented. A 64-year-old woman was diagnosed with AIP in 2007. The symptoms resolved with prednisolone (PSL). Although the dose of PSL was tapered to 7.5 mg/day for maintenance, serum IgG4 levels remained high. She suddenly presented with subcutaneous bleeding in 2015. Her activated partial thromboplastin time was prolonged (80.0 s). A mixing test showed an inhibitor pattern, factor VIII (FVIII) activity was less than 1%, and FVIII inhibitor was 290 BU/mL. She was diagnosed with AHA. Her serum IgG4 was elevated to 133 mg/dL. She was treated first with PSL alone, but she developed bladder tamponade. Cyclophosphamide and activated prothrombin complex concentrate were combined with PSL. She then achieved hemostasis, and FVIII inhibitor disappeared. FVIII inhibitor had been detected since PSL was tapered and AHA recurred two months later. An enzyme-linked immunosorbent assay showed that the inhibitor was mainly IgG4 and IgG1. This case suggests that elevation of IgG4 may be associated with the development of both AHA and AIP.</abstract><cop>Tokyo</cop><pub>Springer Japan</pub><pmid>29582334</pmid><doi>10.1007/s12185-018-2441-3</doi><tpages>4</tpages><orcidid>https://orcid.org/0000-0002-0403-0122</orcidid></addata></record> |
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| subjects | Bladder Bleeding Case Report Coagulation factors Cyclophosphamide Enzyme-linked immunosorbent assay Factor VIII deficiency Hematology Hemophilia Hemostasis Hemostatics Immunoglobulin G Inhibitors Medicine Medicine & Public Health Oncology Pancreatitis Prednisolone Prothrombin Tamponade Thromboplastin |
| title | Acquired hemophilia A associated with autoimmune pancreatitis with serum IgG4 elevation |
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