Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry

被引：288

作者：

Baudo, Francesco ^{[1
]}

Collins, Peter ^{[2
]}

Huth-Kuehne, Angela ^{[3
,4
]}

Levesque, Herve ^{[5
]}

Marco, Pascual ^{[6
]}

Nemes, Laszlo ^{[7
,8
]}

Pellegrini, Fabio ^{[9
]}

Tengborn, Lilian ^{[10
]}

Knoebl, Paul ^{[11
]}

Aspoeck, G. ^{[12
]}

Heistinger, M. ^{[13
]}

Knobl, P. ^{[14
]}

Makipernaa, A. ^{[15
]}

André, H. ^{[16
]}

Aouba, A. ^{[17
]}

Bellucci, S. ^{[18
]}

Beurrier, P. ^{[19
]}

Borg, J. Y. ^{[20
]}

Darnige, L. ^{[21
]}

Devignes, J. ^{[22
]}

d'Oiron, R. ^{[23
]}

Gautier, P. ^{[24
]}

Gay, V. ^{[25
]}

Girault, S. ^{[26
]}

Gruel, Y. ^{[27
]}

Guerin, V. ^{[28
]}

Hézard, N. ^{[29
]}

Khellaf, M. ^{[30
]}

Koenig, M. ^{[31
]}

Lévesque, H. ^{[32
]}

Lifermann, F. ^{[33
]}

Marlu, R. ^{[34
]}

Ninet, J. ^{[35
]}

Peynet, J. ^{[36
]}

Quemeneur, T. ^{[37
]}

Rothschild, C. ^{[38
]}

Schleinitz, N. ^{[39
]}

Sigaud, M. ^{[40
]}

Trouillier, S. ^{[41
]}

Voisin, S. ^{[42
]}

Giebl, A. ^{[43
]}

Holstein, K. ^{[44
]}

Huth-Kuhne, A. ^{[45
]}

Loreth, R. M. ^{[46
]}

Steigerwald, U. ^{[47
]}

Tiede, A. ^{[48
]}

Theodossiades, G. ^{[49
]}

Nemes, L. ^{[50
]}

Radvanyi, G. ^{[51
]}

Schlammadinger, A. ^{[52
]}

机构：

[1] Osped Niguarda Ca Granda, Thrombosis & Hemostasis Unit, Piazza Osped Maggiore 3, I-20162 Milan, Italy

[2] Cardiff Univ, Sch Med, Univ Wales Hosp, Arthur Bloom Haemophilia Ctr, Cardiff, S Glam, Wales

[3] SRH Kurpfalzkrankenhaus Heidelberg GmbH, Heidelberg, Germany

[4] Hemophilia Ctr, Heidelberg, Germany

[5] Rouen Univ Hosp, Dept Internal Med, Rouen, France

[6] Hosp Gen Univ, Serv Hematol, Unidad Hemostasia & Trombosis, Alicante, Spain

[7] Natl Hemophilia Ctr, Budapest, Hungary

[8] State Hlth Ctr, Hemostasis Dept, Budapest, Hungary

[9] Consorzio Mario Negri Sud, Dept Clin Pharmacol & Epidemiol, Biostat Unit, Chieti, Italy

[10] Skane Univ Hosp, Clin Coagulat Res Unit, Malmo, Sweden

[11] Med Univ Vienna, Dept Med 1, Div Hematol & Hemostasis, Vienna, Austria

[12] Klinikum Wels Grieskirchen, A-4600 Wels, Austria

[13] LKH Klagenfurt, Med Abt 1, A-9020 Klagenfurt, Austria

[14] Med Univ Wien, Klin Innere Med 1, Abt Hamatol & Hamostaseol, A-1090 Vienna, Austria

[15] Helsinki Univ Hosp, Dept Haematol Coagulat Disorders, Helsinki 00029, Finland

[16] Hop Europeen Georges Pompidou, Serv Med Interne, F-75015 Paris, France

[17] Hop Necker Enfants Malad, Dept Haematol, F-75015 Paris, France

[18] Hop Lariboisiere, Serv Hematol Biol, F-75010 Paris, France

[19] CHU Angers, Ctr Traitement Hemophile, F-49933 Angers, France

[20] Rouen Univ Hosp, Haematol Lab, F-76031 Rouen, France

[21] Hop Europeen Georges Pompidou, Serv Hematol Biol, F-75908 Paris, France

[22] Ctr Hosp Univ Nancy, Serv Hematol Biol Hemostase, F-545111 Nancy, France

[23] APHP Bicetre Univ Hosp, Hemophilia Ctr, F-94720 Le Kremlin Bicetre, France

[24] CHU, Lab Hematol, CRTH, F-14033 Caen, France

[25] Hop Chambery, Serv Hemovigilance, F-73011 Chambery, France

[26] CHU Dupuytren, F-87000 Limoges, France

[27] Univ Hosp Tours, Hemostasis, F-37044 Tours, France

[28] Hop Pellegrin, Hematol Lab, F-33076 Bordeaux, France

[29] CHU Robert Debre, Lab Hematol, F-51092 Reims, France

[30] CHU Henri Mondor, Serv Med Interne, F-94010 Creteil, France

[31] CHU St Etienne, Med Interne, F-42055 St Etienne, France

[32] Rouen Univ Hosp, Dept Internal Med, F-76031 Rouen, France

[33] Ctr Hosp Gen, Dept Med Interne, F-40100 Dax, France

[34] CHU Grenoble, Lab Hematol, F-38700 La Tonche, France

[35] Hop Edouard Herriot, Serv Med Interne, F-69437 Lyon, France

[36] Ctr Hosp de Versailles, Ctr Traitement Hemophilie, F-78157 Le Chesnay, France

[37] SCE Med Interne & Nephrol, F-59322 Valenciennes, France

[38] Hop Necker Enfants Malad, Hemophilia Ctr, F-75015 Paris, France

[39] CHU Conception, Serv Med Interne, F-13385 Marseille, France

[40] CHU Nantes, Ctr Traitement Hemophilie, Lab Hematol, F-44093 Nantes, France

[41] CHU Gabriel Montpied, Serv Med Interne, F-6300 Clermont Ferrand, France

[42] Hop Rangueil, Lab Hematol, F-31059 Toulouse 9, France

[43] Klinikum Univ Munchen, Klin Anastheseol, F-81366 Munich, Germany

[44] Univ Krankenhaus Hamburg Eppendorf, Gerinnung, F-20246 Hamburg, Germany

[45] SRH Kurpfalzkrankenhaus Heidelberg, Innere Med Hamophiliezentrum, D-69123 Heidelberg, Germany

[46] Westpfalz Klinikum GmbH, Med Klin 3 Klin Hamostaseol, D-67655 Kaiserslautern, Germany

[47] Univ Klinikum Wurzburg, Zentrallabor Gerinnungsambulanz, D-97080 Wurzburg, Germany

[48] Hannover Med Sch, Hamatol Onkol, D-30625 Hannover, Germany

[49] Hippocrat Hosp, Haemophilia Ctr, GR-11527 Athens, Greece

[50] State Hlth Ctr, H-1134 Budapest, Hungary

来源：

BLOOD | 2012年 / 120卷 / 01期

关键词：

RECOMBINANT FACTOR VIIA; UNITED-KINGDOM; FACTOR-IX; INHIBITORS; DIAGNOSIS; SURGERY; THERAPY; FEIBA;

D O I：

10.1182/blood-2012-02-408930

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%). (Blood. 2012;120(1):39-46)

引用

页码：39 / 46

页数：8

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