Hemostatic assessment of combined anticoagulant therapy using warfarin and prothrombin complex concentrates in a case of severe protein C deficiency

Kenichi Ogiwara, Keiji Nogami, Kuniyoshi Mizumachi, Takashi Nakagawa, Nozomi Noda, Shoichi Ohga, Midori Shima

研究成果: ジャーナルへの寄稿記事

抄録

Patients with severe congenital protein (P)C deficiency require long-term anticoagulant management. Recombinant PC concentrates for prophylactic use are not available in Japan; prothrombin complex concentrates (PCC), containing factors (F)II, VII, IX, X, and PC (PPSB-HT®), have been used ‘off-label’ in a few patients. We investigated the combined use of prophylactic PCC and Warfarin (VKA; PT-INR 2.0–2.5) in a severely PC-deficient patient in whom VKA alone did not prevent recurrent purpura. Plasma VKA-dependent factor levels and global PC function (Thrombopath®) were assessed. Plasma activity levels of FII/FVII/FIX/FX post-infusion of PCC (6.3 unit/kg) increased 35/27/27/35 (initial level) to 59/60/38/83 IU/dl, respectively. FVII:C and FIX:C rapidly returned to baseline levels 12–24 h post-infusion, but FII:C and FX:C returned more slowly. PC antigen (< 5%) increased to ~ 15%, followed by return to baseline levels 24 h post-infusion. Global PC function was very low (%PiCi 24%), but improved post-PCC infusion. This potential was slightly detectable even at an undetectable PC level. At day 3, high levels of d-dimer and FDP were observed without thrombotic event, but these improved post-infusion. Although PCC restored VKA-dependent coagulation factors, PC contained in PCC significantly improved global anticoagulation, and was clinically beneficial in this severely deficient patient.

元の言語英語
ページ(範囲)650-656
ページ数7
ジャーナルInternational journal of hematology
109
発行部数6
DOI
出版物ステータス出版済み - 6 20 2019

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Protein C Deficiency
Hemostatics
Warfarin
Anticoagulants
Therapeutics
Purpura
Blood Coagulation Factors
International Normalized Ratio
prothrombin complex concentrates
Japan
Antigens

All Science Journal Classification (ASJC) codes

  • Hematology

これを引用

Hemostatic assessment of combined anticoagulant therapy using warfarin and prothrombin complex concentrates in a case of severe protein C deficiency. / Ogiwara, Kenichi; Nogami, Keiji; Mizumachi, Kuniyoshi; Nakagawa, Takashi; Noda, Nozomi; Ohga, Shoichi; Shima, Midori.

:: International journal of hematology, 巻 109, 番号 6, 20.06.2019, p. 650-656.

研究成果: ジャーナルへの寄稿記事

Ogiwara, Kenichi ; Nogami, Keiji ; Mizumachi, Kuniyoshi ; Nakagawa, Takashi ; Noda, Nozomi ; Ohga, Shoichi ; Shima, Midori. / Hemostatic assessment of combined anticoagulant therapy using warfarin and prothrombin complex concentrates in a case of severe protein C deficiency. :: International journal of hematology. 2019 ; 巻 109, 番号 6. pp. 650-656.
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title = "Hemostatic assessment of combined anticoagulant therapy using warfarin and prothrombin complex concentrates in a case of severe protein C deficiency",
abstract = "Patients with severe congenital protein (P)C deficiency require long-term anticoagulant management. Recombinant PC concentrates for prophylactic use are not available in Japan; prothrombin complex concentrates (PCC), containing factors (F)II, VII, IX, X, and PC (PPSB-HT{\circledR}), have been used ‘off-label’ in a few patients. We investigated the combined use of prophylactic PCC and Warfarin (VKA; PT-INR 2.0–2.5) in a severely PC-deficient patient in whom VKA alone did not prevent recurrent purpura. Plasma VKA-dependent factor levels and global PC function (Thrombopath{\circledR}) were assessed. Plasma activity levels of FII/FVII/FIX/FX post-infusion of PCC (6.3 unit/kg) increased 35/27/27/35 (initial level) to 59/60/38/83 IU/dl, respectively. FVII:C and FIX:C rapidly returned to baseline levels 12–24 h post-infusion, but FII:C and FX:C returned more slowly. PC antigen (< 5{\%}) increased to ~ 15{\%}, followed by return to baseline levels 24 h post-infusion. Global PC function was very low ({\%}PiCi 24{\%}), but improved post-PCC infusion. This potential was slightly detectable even at an undetectable PC level. At day 3, high levels of d-dimer and FDP were observed without thrombotic event, but these improved post-infusion. Although PCC restored VKA-dependent coagulation factors, PC contained in PCC significantly improved global anticoagulation, and was clinically beneficial in this severely deficient patient.",
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AU - Ogiwara, Kenichi

AU - Nogami, Keiji

AU - Mizumachi, Kuniyoshi

AU - Nakagawa, Takashi

AU - Noda, Nozomi

AU - Ohga, Shoichi

AU - Shima, Midori

PY - 2019/6/20

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N2 - Patients with severe congenital protein (P)C deficiency require long-term anticoagulant management. Recombinant PC concentrates for prophylactic use are not available in Japan; prothrombin complex concentrates (PCC), containing factors (F)II, VII, IX, X, and PC (PPSB-HT®), have been used ‘off-label’ in a few patients. We investigated the combined use of prophylactic PCC and Warfarin (VKA; PT-INR 2.0–2.5) in a severely PC-deficient patient in whom VKA alone did not prevent recurrent purpura. Plasma VKA-dependent factor levels and global PC function (Thrombopath®) were assessed. Plasma activity levels of FII/FVII/FIX/FX post-infusion of PCC (6.3 unit/kg) increased 35/27/27/35 (initial level) to 59/60/38/83 IU/dl, respectively. FVII:C and FIX:C rapidly returned to baseline levels 12–24 h post-infusion, but FII:C and FX:C returned more slowly. PC antigen (< 5%) increased to ~ 15%, followed by return to baseline levels 24 h post-infusion. Global PC function was very low (%PiCi 24%), but improved post-PCC infusion. This potential was slightly detectable even at an undetectable PC level. At day 3, high levels of d-dimer and FDP were observed without thrombotic event, but these improved post-infusion. Although PCC restored VKA-dependent coagulation factors, PC contained in PCC significantly improved global anticoagulation, and was clinically beneficial in this severely deficient patient.

AB - Patients with severe congenital protein (P)C deficiency require long-term anticoagulant management. Recombinant PC concentrates for prophylactic use are not available in Japan; prothrombin complex concentrates (PCC), containing factors (F)II, VII, IX, X, and PC (PPSB-HT®), have been used ‘off-label’ in a few patients. We investigated the combined use of prophylactic PCC and Warfarin (VKA; PT-INR 2.0–2.5) in a severely PC-deficient patient in whom VKA alone did not prevent recurrent purpura. Plasma VKA-dependent factor levels and global PC function (Thrombopath®) were assessed. Plasma activity levels of FII/FVII/FIX/FX post-infusion of PCC (6.3 unit/kg) increased 35/27/27/35 (initial level) to 59/60/38/83 IU/dl, respectively. FVII:C and FIX:C rapidly returned to baseline levels 12–24 h post-infusion, but FII:C and FX:C returned more slowly. PC antigen (< 5%) increased to ~ 15%, followed by return to baseline levels 24 h post-infusion. Global PC function was very low (%PiCi 24%), but improved post-PCC infusion. This potential was slightly detectable even at an undetectable PC level. At day 3, high levels of d-dimer and FDP were observed without thrombotic event, but these improved post-infusion. Although PCC restored VKA-dependent coagulation factors, PC contained in PCC significantly improved global anticoagulation, and was clinically beneficial in this severely deficient patient.

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