Comprehensive blood coagulation potential in patients with acquired hemophilia A: retrospective analyses of plasma samples obtained from nationwide centers across Japan
Global coagulation potential was assessed in 59 patients with acquired hemophilia A (PwAHA) by clot waveform analysis (CWA) and/or thrombin and plasmin generation assay. Relationships between factor VIII activity (FVIII:C) and the parameters from CWA and T/P-GA in patients with congenital HA were co...
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Veröffentlicht in: | International journal of hematology 2022-02, Vol.115 (2), p.163-172 |
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container_title | International journal of hematology |
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creator | Takeyama, Masahiro Sasai, Kana Matsumoto, Tomoko Furukawa, Shoko Ogiwara, Kenichi Yada, Koji Onishi, Tomoko Shima, Midori Nogami, Keiji |
description | Global coagulation potential was assessed in 59 patients with acquired hemophilia A (PwAHA) by clot waveform analysis (CWA) and/or thrombin and plasmin generation assay. Relationships between factor VIII activity (FVIII:C) and the parameters from CWA and T/P-GA in patients with congenital HA were compared by grading coagulation potential related to FVIII:C: T1 (FVIII:C |
doi_str_mv | 10.1007/s12185-021-03249-w |
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Relationships between factor VIII activity (FVIII:C) and the parameters from CWA and T/P-GA in patients with congenital HA were compared by grading coagulation potential related to FVIII:C: T1 (FVIII:C < 1 IU/dL), T2 (1 ≤ , ≤ 5 IU/dL), T3 (5 < , 12 ≤ IU/dL), and T4 (12 < , ≤ 50 IU/dL). The median FVIII:C and inhibitor titers in PwAHA on admission were 3.3 IU/dL and 63.0 BU/mL, respectively, but global coagulation parameters corresponded to T1 or less. Median FVIII:C levels during follow-up in PwAHA were 1.7–9.6–6.7–40.0–21.7 IU/dL on days 0–14–28–56–93, respectively. CWA-based data corresponded to less than T2 until day 28, but more closely reflected FVIII:C after day 56. Peak thrombin was severely low (near T1) until day 28 and improved modestly after day 56 but remained less than T2. Peak plasmin was lower than T1 until day 56, and returned to T4 on day 93. In conclusion, global coagulation function in PwAHA was impaired to a greater extent than could be anticipated from assays of FVIII:C, until approximately 1 month after immunosuppression and treatment with FVIII-bypassing agents.</description><identifier>ISSN: 0925-5710</identifier><identifier>EISSN: 1865-3774</identifier><identifier>DOI: 10.1007/s12185-021-03249-w</identifier><identifier>PMID: 34724152</identifier><language>eng</language><publisher>Singapore: Springer Singapore</publisher><subject>Adolescent ; Adult ; Aged ; Aged, 80 and over ; Blood Coagulation ; Blood Coagulation Tests ; Child ; Coagulation ; Coagulation factors ; Female ; Hematology ; Hemophilia ; Hemophilia A - blood ; Hemophilia A - epidemiology ; Humans ; Immunosuppression ; Japan - epidemiology ; Male ; Medicine ; Medicine & Public Health ; Middle Aged ; Oncology ; Original Article ; Parameters ; Patients ; Plasmin ; Retrospective Studies ; Thrombin ; Waveforms ; Young Adult</subject><ispartof>International journal of hematology, 2022-02, Vol.115 (2), p.163-172</ispartof><rights>Japanese Society of Hematology 2021</rights><rights>2021. Japanese Society of Hematology.</rights><rights>Japanese Society of Hematology 2021.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c375t-e6324137bed813b9f788fa1811e993f8b7e715a69b04936c5c9826c8e0b870353</citedby><cites>FETCH-LOGICAL-c375t-e6324137bed813b9f788fa1811e993f8b7e715a69b04936c5c9826c8e0b870353</cites><orcidid>0000-0002-9348-9815</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/s12185-021-03249-w$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s12185-021-03249-w$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,777,781,27905,27906,41469,42538,51300</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/34724152$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Takeyama, Masahiro</creatorcontrib><creatorcontrib>Sasai, Kana</creatorcontrib><creatorcontrib>Matsumoto, Tomoko</creatorcontrib><creatorcontrib>Furukawa, Shoko</creatorcontrib><creatorcontrib>Ogiwara, Kenichi</creatorcontrib><creatorcontrib>Yada, Koji</creatorcontrib><creatorcontrib>Onishi, Tomoko</creatorcontrib><creatorcontrib>Shima, Midori</creatorcontrib><creatorcontrib>Nogami, Keiji</creatorcontrib><title>Comprehensive blood coagulation potential in patients with acquired hemophilia A: retrospective analyses of plasma samples obtained from nationwide centers across Japan</title><title>International journal of hematology</title><addtitle>Int J Hematol</addtitle><addtitle>Int J Hematol</addtitle><description>Global coagulation potential was assessed in 59 patients with acquired hemophilia A (PwAHA) by clot waveform analysis (CWA) and/or thrombin and plasmin generation assay. Relationships between factor VIII activity (FVIII:C) and the parameters from CWA and T/P-GA in patients with congenital HA were compared by grading coagulation potential related to FVIII:C: T1 (FVIII:C < 1 IU/dL), T2 (1 ≤ , ≤ 5 IU/dL), T3 (5 < , 12 ≤ IU/dL), and T4 (12 < , ≤ 50 IU/dL). The median FVIII:C and inhibitor titers in PwAHA on admission were 3.3 IU/dL and 63.0 BU/mL, respectively, but global coagulation parameters corresponded to T1 or less. Median FVIII:C levels during follow-up in PwAHA were 1.7–9.6–6.7–40.0–21.7 IU/dL on days 0–14–28–56–93, respectively. CWA-based data corresponded to less than T2 until day 28, but more closely reflected FVIII:C after day 56. Peak thrombin was severely low (near T1) until day 28 and improved modestly after day 56 but remained less than T2. Peak plasmin was lower than T1 until day 56, and returned to T4 on day 93. In conclusion, global coagulation function in PwAHA was impaired to a greater extent than could be anticipated from assays of FVIII:C, until approximately 1 month after immunosuppression and treatment with FVIII-bypassing agents.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Blood Coagulation</subject><subject>Blood Coagulation Tests</subject><subject>Child</subject><subject>Coagulation</subject><subject>Coagulation factors</subject><subject>Female</subject><subject>Hematology</subject><subject>Hemophilia</subject><subject>Hemophilia A - blood</subject><subject>Hemophilia A - epidemiology</subject><subject>Humans</subject><subject>Immunosuppression</subject><subject>Japan - epidemiology</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Middle Aged</subject><subject>Oncology</subject><subject>Original Article</subject><subject>Parameters</subject><subject>Patients</subject><subject>Plasmin</subject><subject>Retrospective Studies</subject><subject>Thrombin</subject><subject>Waveforms</subject><subject>Young Adult</subject><issn>0925-5710</issn><issn>1865-3774</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNp9kc2OFCEUhYnROG3rC7gwJG7clPJTFOBu0vE3k7jRNaGoW9NMqKIGqOnMG_mY0t2jJi5ckQvfOSeXg9BLSt5SQuS7TBlVoiGMNoSzVjeHR2hDVScaLmX7GG2IZqIRkpIL9CznG0KoJK18ii54K1lLBdugn7s4LQn2MGd_B7gPMQ7YRXu9Blt8nPESC8zF24B9HepdnTI--LLH1t2uPsGA9zDFZe-Dt_jyPU5QUswLuHJ0tLMN9xkyjiNegs2TxdlOSzje9MX6uerHFCc8n_IOfgDsagakXAOqUcZf7WLn5-jJaEOGFw_nFv34-OH77nNz9e3Tl93lVeO4FKWBrv4E5bKHQVHe61EqNVqqKAWt-ah6CZIK2-metJp3TjitWOcUkF5JwgXfojdn3yXF2xVyMZPPDkKwM8Q1GyY047RTVb1Fr_9Bb-Ka6r6V6pgkRItOVYqdqdMyCUazJD_ZdG8oMccezblHU3s0px7NoYpePViv_QTDH8nv4irAz0CuT_M1pL_Z_7H9BQcbrIA</recordid><startdate>20220201</startdate><enddate>20220201</enddate><creator>Takeyama, Masahiro</creator><creator>Sasai, Kana</creator><creator>Matsumoto, Tomoko</creator><creator>Furukawa, Shoko</creator><creator>Ogiwara, Kenichi</creator><creator>Yada, Koji</creator><creator>Onishi, Tomoko</creator><creator>Shima, Midori</creator><creator>Nogami, Keiji</creator><general>Springer Singapore</general><general>Springer Nature B.V</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>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/0000-0002-9348-9815</orcidid></search><sort><creationdate>20220201</creationdate><title>Comprehensive blood coagulation potential in patients with acquired hemophilia A: retrospective analyses of plasma samples obtained from nationwide centers across Japan</title><author>Takeyama, Masahiro ; 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Relationships between factor VIII activity (FVIII:C) and the parameters from CWA and T/P-GA in patients with congenital HA were compared by grading coagulation potential related to FVIII:C: T1 (FVIII:C < 1 IU/dL), T2 (1 ≤ , ≤ 5 IU/dL), T3 (5 < , 12 ≤ IU/dL), and T4 (12 < , ≤ 50 IU/dL). The median FVIII:C and inhibitor titers in PwAHA on admission were 3.3 IU/dL and 63.0 BU/mL, respectively, but global coagulation parameters corresponded to T1 or less. Median FVIII:C levels during follow-up in PwAHA were 1.7–9.6–6.7–40.0–21.7 IU/dL on days 0–14–28–56–93, respectively. CWA-based data corresponded to less than T2 until day 28, but more closely reflected FVIII:C after day 56. Peak thrombin was severely low (near T1) until day 28 and improved modestly after day 56 but remained less than T2. Peak plasmin was lower than T1 until day 56, and returned to T4 on day 93. In conclusion, global coagulation function in PwAHA was impaired to a greater extent than could be anticipated from assays of FVIII:C, until approximately 1 month after immunosuppression and treatment with FVIII-bypassing agents.</abstract><cop>Singapore</cop><pub>Springer Singapore</pub><pmid>34724152</pmid><doi>10.1007/s12185-021-03249-w</doi><tpages>10</tpages><orcidid>https://orcid.org/0000-0002-9348-9815</orcidid></addata></record> |
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subjects | Adolescent Adult Aged Aged, 80 and over Blood Coagulation Blood Coagulation Tests Child Coagulation Coagulation factors Female Hematology Hemophilia Hemophilia A - blood Hemophilia A - epidemiology Humans Immunosuppression Japan - epidemiology Male Medicine Medicine & Public Health Middle Aged Oncology Original Article Parameters Patients Plasmin Retrospective Studies Thrombin Waveforms Young Adult |
title | Comprehensive blood coagulation potential in patients with acquired hemophilia A: retrospective analyses of plasma samples obtained from nationwide centers across Japan |
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