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
Hauptverfasser: Takeyama, Masahiro, Sasai, Kana, Matsumoto, Tomoko, Furukawa, Shoko, Ogiwara, Kenichi, Yada, Koji, Onishi, Tomoko, Shima, Midori, Nogami, Keiji
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container_issue 2
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container_title International journal of hematology
container_volume 115
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 &lt; 1 IU/dL), T2 (1 ≤ , ≤ 5 IU/dL), T3 (5 &lt; , 12 ≤ IU/dL), and T4 (12 &lt; , ≤ 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. 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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 &lt; 1 IU/dL), T2 (1 ≤ , ≤ 5 IU/dL), T3 (5 &lt; , 12 ≤ IU/dL), and T4 (12 &lt; , ≤ 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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