The safety of the combination therapy of recombinant factor VIIa and plasma-derived factor VIIa and factor X for refractory hemorrhage in acquired hemophilia A

被引：1

作者：

Okayama, Yusuke ^{[1
]}

Bingo, Masato ^{[2
]}

Sakatoku, Kazuki ^{[1
]}

Okamura, Hiroshi ^{[1
]}

Nanno, Satoru ^{[1
]}

Nishimoto, Mitsutaka ^{[1
]}

Nakashima, Yasuhiro ^{[1
,3
]}

Koh, Hideo ^{[1
]}

Hino, Masayuki ^{[1
]}

Nakamae, Hirohisa ^{[1
]}

机构：

[1] Osaka Metropolitan Univ, Grad Sch Med, Dept Hematol, Osaka, Japan

[2] Tokyo Med Univ, Dept Lab Med, Tokyo, Japan

[3] Osaka Metropolitan Univ, Grad Sch Med, Dept Hematol, 1-4-3 Asahi machi,Abeno ku, Osaka 5458585, Japan

来源：

BLOOD COAGULATION & FIBRINOLYSIS | 2023年 / 34卷 / 06期

关键词：

acquired hemophilia A; combination therapy; plasma-derived factor VIIa and factor X; recombinant factor VIIa; BYPASSING AGENTS; INHIBITORS; PATIENT;

D O I：

10.1097/MBC.0000000000001243

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Acquired hemophilia A (AHA) is a rare, life-threatening hemorrhagic disease caused by autoantibodies against factor VIII (FVIII), and bypassing agents (BPA) are used to control bleeding. However, some cases need a change of BPA or BPAs given sequentially or in combination for refractory bleeding. A 71-year-old man was admitted with subcutaneous hemorrhage. Laboratory investigations showed prolongation of activated partial thromboplastin time (APTT) and low-coagulation FVIII activity and FVIII inhibitor; we, therefore, diagnosed AHA. He was treated with recombinant factor VIIa (rFVIIa) BPA and prednisolone. However, his symptoms did not improve sufficiently, thus we switched BPA to activated prothrombin complex concentrate. Unfortunately, this was not effective and he suffered hemorrhagic shock. Therefore, we selected rFVIIa, with plasma-derived FVIIa and factor X (pd-FVIIa/FX) as combination therapy, and hemostasis was achieved without thrombosis. This case suggests that the combination of rFVIIa and pd-FVIIa/FX short-term can be well tolerated for refractory hemorrhage in AHA.

引用

页码：419 / 422

页数：4

共 11 条

[1] A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: the FEIBA NovoSeven Comparative (FENOC) Study [J].

Astermark, Jan ;

Donfield, Sharyne M. ;

DiMichele, Donna M. ;

Gringeri, Alessandro ;

Gilbert, Steven A. ;

Waters, Jennifer ;

Berntorp, Erik .

BLOOD, 2007, 109 (02) :546-551

[2] Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry [J].

Baudo, Francesco ;

Collins, Peter ;

Huth-Kuehne, Angela ;

Levesque, Herve ;

Marco, Pascual ;

Nemes, Laszlo ;

Pellegrini, Fabio ;

Tengborn, Lilian ;

Knoebl, Paul ;

Aspoeck, G. ;

Heistinger, M. ;

Knobl, P. ;

Makipernaa, A. ;

André, H. ;

Aouba, A. ;

Bellucci, S. ;

Beurrier, P. ;

Borg, J. Y. ;

Darnige, L. ;

Devignes, J. ;

d'Oiron, R. ;

Gautier, P. ;

Gay, V. ;

Girault, S. ;

Gruel, Y. ;

Guerin, V. ;

Hézard, N. ;

Khellaf, M. ;

Koenig, M. ;

Lévesque, H. ;

Lifermann, F. ;

Marlu, R. ;

Ninet, J. ;

Peynet, J. ;

Quemeneur, T. ;

Rothschild, C. ;

Schleinitz, N. ;

Sigaud, M. ;

Trouillier, S. ;

Voisin, S. ;

Giebl, A. ;

Holstein, K. ;

Huth-Kuhne, A. ;

Loreth, R. M. ;

Steigerwald, U. ;

Tiede, A. ;

Theodossiades, G. ;

Nemes, L. ;

Radvanyi, G. ;

Schlammadinger, A. .

BLOOD, 2012, 120 (01) :39-46

[3] Unresponsiveness to factor VIII inhibitor bypassing agents during haemostatic treatment for life-threatening massive bleeding in a patient with haemophilia A and a high responding inhibitor [J].

Hayashi, T ;

Tanaka, I ;

Shima, M ;

Yoshida, K ;

Fukuda, K ;

Sakurai, Y ;

Matsumoto, T ;

Giddings, JC ;

Yoshioka, A .

HAEMOPHILIA, 2004, 10 (04) :397-400

[4] Parallel use of by-passing agents in haemophilia with inhibitors: a critical review [J].

Ingerslev, Jorgen ;

Sorensen, Benny .

BRITISH JOURNAL OF HAEMATOLOGY, 2011, 155 (02) :256-262

[5] Plasma-derived factors VIIa and X mixtures (Byclot®) significantly improve impairment of coagulant potential ex vivo in plasmas from acquired hemophilia A patients [J].

Ochi, Satoshi ;

Takeyama, Masahiro ;

Shima, Midori ;

Nogami, Keiji .

INTERNATIONAL JOURNAL OF HEMATOLOGY, 2020, 111 (06) :779-785

[6] A phase III clinical trial of a mixture agent of plasma-derived factor VIIa and factor X (MC710) in haemophilia patients with inhibitors [J].

Shinkoda, Y. ;

Shirahata, A. ;

Fukutake, K. ;

Takamatsu, J. ;

Shima, M. ;

Hanabusa, H. ;

Mugishima, H. ;

Takedani, H. ;

Kawasugi, K. ;

Taki, M. ;

Matsushita, T. ;

Tawa, A. ;

Nogami, K. ;

Higasa, S. ;

Kosaka, Y. ;

Fujii, T. ;

Sakai, M. ;

Migita, M. ;

Uchiba, M. ;

Kawakami, K. ;

Sameshima, K. ;

Ohashi, Y. ;

Saito, H. .

HAEMOPHILIA, 2017, 23 (01) :59-66

[7] Perioperative safety and haematostatic efficacy of a new bypassing agent pd-FVIIa/FX (Byclot) in haemophilia patients with high-responding type inhibitors [J].

Shirayama, Rie ;

Takedani, Hideyuki ;

Chikasawa, Yushi ;

Ishiguro, Akira ;

Ishimura, Masataka ;

Isobe, Kiyotaka ;

Uchiba, Mitsuhiro ;

Ogata, Yoshiyasu ;

Kakuda, Harumi ;

Kusuhara, Koichi ;

Shirahata, Akira .

BLOOD COAGULATION & FIBRINOLYSIS, 2019, 30 (08) :385-392

[8] Major orthopaedic surgery for a haemophilia patient with inhibitors using a new bypassing agent [J].

Takedani, H. ;

Hirose, J. ;

Minamoto, F. ;

Kubota, M. ;

Kinkawa, J. ;

Noguchi, M. .

HAEMOPHILIA, 2016, 22 (05) :E459-E461

[9] Clinical evaluation of bleeds and response to haemostatic treatment in patients with acquired haemophilia: A global expert consensus statement [J].

Tiede, Andreas ;

Giangrande, Paul ;

Teitel, Jerome ;

Amano, Kagehiro ;

Benson, Gary ;

Nemes, Laszlo ;

Jimenez-Yuste, Victor ;

d'Oiron, Roseline ;

El Fegoun, Soraya Benchikh ;

Kessler, Craig M. .

HAEMOPHILIA, 2019, 25 (06) :969-978

[10] A novel therapeutic approach combining human plasma-derived Factors VIIa and X for haemophiliacs with inhibitors:: evidence of a higher thrombin generation rate in vitro and more sustained haemostatic activity in vivo than obtained with Factor VIIa alone [J].

Tomokiyo, K ;

Nakatomi, Y ;

Araki, T ;

Teshima, K ;

Nakano, H ;

Nakagaki, T ;

Miyamoto, S ;

Funatsu, A ;

Iwanaga, S .

VOX SANGUINIS, 2003, 85 (04) :290-299

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