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


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2019-04-08

AUTHORS

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

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®), 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. More... »

PAGES

650-656

References to SciGraph publications

  • 2016-08-18. Pediatric thromboembolism: a national survey in Japan in INTERNATIONAL JOURNAL OF HEMATOLOGY
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    http://scigraph.springernature.com/pub.10.1007/s12185-019-02645-7

    DOI

    http://dx.doi.org/10.1007/s12185-019-02645-7

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    https://app.dimensions.ai/details/publication/pub.1113301202

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/30963470


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