Low dose of aPCC after the initial treatment in acquired haemophilia A is useful to reduce bleeding relapses: Data from the FAIR registry.


BACKGROUND:Bypassing agents are the first line therapy in patients with acquired haemophilia A (AHA). Activated prothrombin complex concentrate (aPCC) proved to be effective as initial treatment, but 20% of patients (pts) had relapses. aPCC as short-term prophylaxis to reduce subsequent bleeds is still not clear. AIM:To evaluate whether a short-term prophylaxis with low dose of aPCC can reduce bleeding relapses after initial AHA treatment, maintaining safety. METHODS:The FAIR Registry is a retrospective-prospective study started on December 2012, that collected data on all pts with AHA treated with aPCC in 12 Italian Haemophilia Centers. All statistical analyses were carried out in the 56 pts included in the registry. RESULTS:31 retrospective and 25 prospective pts were evaluated.101 bleeds requiring treatment were reported, 84.1% spontaneous, 71.3% involving muscles or skin. Major bleeds were 38,6%. Low-dose aPCC as short-term prophylaxis was started after the first resolved episode in 15/56 pts, 58% of whom prospective, in a mean dose of 54.2 ± 23.0 IU/kg, higher (61.4 ± 23.4 IU/kg) in the prospective group than in the retrospective one (44.3 ± 19.7 IU/kg) and it was continued up to a mean of 20.5 ± 17.6 days, similar in both groups. A total of 32 bleeding relapses were reported, 87.5% in the retrospective group. Only 9.4% occurred during short-term prophylaxis (p < 0.05). In our Registry no thromboembolic events were found. CONCLUSION:Initial AHA treatment with aPCC proved to be highly effective, but a consecutive low dose as short-term prophylaxis seems to demonstrate a significant reduction in bleeding relapses maintaining safety.


Thromb Res


Thrombosis research


Zanon E,Pasca S,Siragusa S,Napolitano M,Santoro C,Mameli L,Rocino A,FAIR Study Group.




Has Abstract


2019-02-01 00:00:00












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    authors: Vucelic D,Jesic R,Jovicic S,Zivotic M,Grubor N,Trajkovic G,Canic I,Elezovic I,Antovic A

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  • Prolonged low-molecular-weight heparin use during pregnancy and subsequent bone mineral density.

    abstract:INTRODUCTION:In contrast to unfractionated heparin (UFH), use of low-molecular weight heparin (LMWH) during pregnancy has not been reported to be associated with a significant decrease in bone mineral density (BMD). The aim of this study was to investigate whether long-term use of LMWH during pregnancy is associated wi...

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  • Protective effects of a specific platelet activating factor (PAF) antagonist, WEB 2086, in traumatic shock.

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    authors: Stahl GL,Bitterman H,Lefer AM

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    authors: Miyamoto S,Ogawa H,Soejima H,Takazoe K,Kajiwara I,Shimomura H,Sakamoto T,Yoshimura M,Kugiyama K,Yasue H,Ozaki Y

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  • Plasma levels of t-PA free PAI-1 and a complex of t-PA with PAI-1 in human males and females at various ages.

    abstract::The concentration of tissue plasminogen activator (t-PA), plasminogen activator inhibitor 1 (PAI-1) and the complex of t-PA and PAI-1 (t-PA-PAI-1 complex) were measured using an enzyme immunoassay, where the first antibody was monoclonal antibody against PAI-1 and the second one was polyclonal anti-t-PA Fab' antibody....

    journal_title:Thrombosis research

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    authors: Takada Y,Takada A

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    authors: Yang Y,Wang C,Jin L,Chen G,Li C,Qi K,Kong D,Wang Y,Song M,Ma L

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    authors: Toulon P,Abecassis L,Smahi M,Ternisien C

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    authors: Ohta S,Niiya K,Sakuragawa N,Fuse H

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    authors: Vallier L,Bouriche T,Bonifay A,Judicone C,Bez J,Franco C,Guervilly C,Hisada Y,Mackman N,Houston R,Poncelet P,Dignat-George F,Lacroix R

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    authors: Kaiser B,Hoppensteadt DA,Jeske W,Wun TC,Fareed J

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  • Clinical evaluation of a pasteurized factor XIII concentrate administration in Henoch-Schönlein purpura. Japanese Pediatric Group.

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    pub_type: 杂志文章


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    journal_title:Thrombosis research

    pub_type: 杂志文章


    authors: van den Herik EG,Cheung EY,de Lau LM,den Hertog HM,Leebeek FW,Dippel DW,Koudstaal PJ,de Maat MP

    更新日期:2012-06-01 00:00:00

  • Interactions between sodium salicylate and acetyl salicylic acid evaluated using ADP induced platelet aggregation and bleeding time.

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    journal_title:Thrombosis research

    pub_type: 杂志文章


    authors: Ring T,Dyerberg J

    更新日期:1985-06-15 00:00:00

  • Parallel characterization of bovine von Willebrand protein (factor VIII-associated protein) by light scattering and SDS gel electrophoresis.

    abstract::Highly purified bovine von Willebrand protein (vWP) has been studied with two complementary techniques. Light scattering measurements on different samples give weight average molecular weights in the range from 7 to 13 million, radius of gyration between 75 and 140 nm and diffusion coefficient near 4.15 X 10(-8) cm2/s...

    journal_title:Thrombosis research

    pub_type: 杂志文章


    authors: Binnie C,Wagner RH,Cooper H,Hermans J

    更新日期:1985-11-15 00:00:00

  • Effect of fibrinogen, fibrin, and fibrin degradation products on transendothelial migration of leukocytes.

    abstract::In spite of numerous studies on the involvement of fibrinogen in transendothelial migration of leukocytes and thereby inflammation, there is still no clear understanding of which fibrin(ogen) species can stimulate leukocyte transmigration. Although we have previously proposed that interaction of fibrin with the VLDL r...

    journal_title:Thrombosis research

    pub_type: 杂志文章


    authors: Yakovlev S,Medved L

    更新日期:2018-02-01 00:00:00

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    pub_type: 杂志文章


    authors: Carnelli V,Gomperts ED,Friedman A,Aledort L,Hilgartner M,Dietrich S,Fedor EJ

    更新日期:1987-06-15 00:00:00

  • Impaired clot lysis by rt-PA catalyzed mini-plasminogen activation.

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    journal_title:Thrombosis research

    pub_type: 杂志文章


    authors: Duboscq C,Genoud V,Parborell MF,Kordich LC

    更新日期:1997-06-15 00:00:00