Induction of tolerance after combined immunosuppression and -adsorption in two patients with acquired haemophilia after severe haemorrhages controlled by sequential administration of rFVIIa and FEIBA

Marcus Stockschläder; Leilani Ruf; Anne Linderer; Thomas Schroeder; Wolfram T. Knoefel; Rainer Haas; Günther Giers; Andrea Gerhardt; Christoph Sucker; Rainer B. Zotz; Rüdiger E. Scharf

doi:10.1160/TH08-11-0716

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 2009; 101(03): 586-590
DOI: 10.1160/TH08-11-0716

Case Report

Schattauer GmbH

Induction of tolerance after combined immunosuppression and -adsorption in two patients with acquired haemophilia after severe haemorrhages controlled by sequential administration of rFVIIa and FEIBA

Marcus Stockschläder

¹Department of Haemostasis and Transfusion Medicine, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Leilani Ruf

²Department of Haematology, Oncology, and Clinical Immunology, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Anne Linderer

³Department of General Surgery, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Thomas Schroeder

²Department of Haematology, Oncology, and Clinical Immunology, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Wolfram T. Knoefel

³Department of General Surgery, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Rainer Haas

²Department of Haematology, Oncology, and Clinical Immunology, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Günther Giers

¹Department of Haemostasis and Transfusion Medicine, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Andrea Gerhardt

¹Department of Haemostasis and Transfusion Medicine, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Christoph Sucker

¹Department of Haemostasis and Transfusion Medicine, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Rainer B. Zotz

¹Department of Haemostasis and Transfusion Medicine, Heinrich Heine University Medical Center, Düsseldorf, Germany

,

Rüdiger E. Scharf

¹Department of Haemostasis and Transfusion Medicine, Heinrich Heine University Medical Center, Düsseldorf, Germany

References
1 Gilles JG, Jacquemin MG, Saint-Remy JM. Factor VIII inhibitors. Thromb Haemost 1997; 78: 641-646.
2 Cohen AJ, Kessler CM. Acquired inhibitors. Bail-lieres Clin Haematol 1996; 9: 331-354.
3 Sallah S, Wan JY. Inhibitors against factor VIII in patients with cancer. Analysis of 41 patients. Cancer 2001; 91: 1067-1074.
4 Hauser I, Schneider B, Lechner K. Post-partum factor VIII inhibitors. A review of the literature with special reference to the value of steroid and immunosuppressive treatment. Thromb Haemost 1995; 73: 1-5.
5 Collins PW, Hirsch S, Baglin TP. et al. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation. Blood 2007; 109: 1870-1877.
6 Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost 1981; 45: 200-203.
7 Morrison AE, Ludlam CA, Kessler C. Use of porcine factor VIII in the treatment of patients with acquired hemophilia. Blood 1993; 81: 1513-1520.
8 Delgado J, Jimenez-Yuste V, Hernandez-Navarro F. et al. Acquired haemophilia: review and meta-analysis focused on therapy and prognostic factors. Br J Haematol 2003; 121: 21-35.
9 Biggs R, Austen DE, Denson KW. et al. The mode of action of antibodies which destroy factor VIII. II. Antibodies which give complex concentration graphs. Br J Haematol 1972; 23: 137-155.
10 Brackmann HH, Oldenburg J, Schwaab R. Immune tolerance for the treatment of factor VIII inhibitors--twenty years’ ‘bonn protocol‘. Vox Sang 1996; 70 (Suppl. 01) 30-35.
11 Negrier C, Goudemand J, Sultan Y. et al. Multi-center retrospective study on the utilization of FEIBA in France in patients with factor VIII and factor IX inhibitors. French FEIBA Study Group. Factor Eight Bypassing Activity. Thromb Haemost 1997; 77: 1113-1119.
12 Sallah S. Treatment of acquired haemophilia with factor eight inhibitor bypassing activity. Haemophilia 2004; 10: 169-173.
13 Ehrlich HJ, Henzl MJ, Gomperts ED. Safety of factor VIII inhibitor bypass activity (FEIBA): 10-year compilation of thrombotic adverse events. Haemophilia 2002; 8: 83-90.
14 Parameswaran R, Shapiro AD, Gill JC. et al. Dose effect and efficacy of rFVIIa in the treatment of haemophilia patients with inhibitors: analysis from the Hemophilia and Thrombosis Research Society Registry. Haemophilia 2005; 11: 100-106.
15 Astermark J, Donfield SM, DiMichele DM. et al. A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: the FEIBA Novo-Seven Comparative (FENOC) Study. Blood 2007; 109: 546-551.
16 Hay CR, Negrier C, Ludlam CA. The treatment of bleeding in acquired haemophilia with recombinant factor VIIa: a multicentre study. Thromb Haemost 1997; 78: 1463-1467.
17 Guillet B, Pinganaud C, Proulle V. et al. Myocardial infarction occurring in a case of acquired haemophilia during the treatment course with recombinant activated factor VII. Thromb Haemost 2002; 88: 698-699.
18 Hedner U, Erhardtsen E. Potential role of recombinant factor VIIa as a hemostatic agent. Clin Adv Hematol Oncol 2003; 1: 112-119.
19 Astermark J, Donfield SM, DiMichele DM. et al. A randomized comparison of bypassing agents in hemophilia complicated by an inhibitor: the FEIBA Novo-Seven Comparative (FENOC) Study. Blood 2007; 109: 546-551.
20 Hoffman M, Monroe III DM. The action of high-dose factor VIIa (FVIIa) in a cell-based model of hemostasis. Dis Mon 2003; 49: 14-21.
21 Turecek PL, Varadi K, Gritsch H. et al. FEIBA: mode of action. Haemophilia 2004; 10 (Suppl. 02) 3-9.
22 Key NS, Christie B, Henderson N. et al. Possible synergy between recombinant factor VIIa and prothrombin complex concentrate in hemophilia therapy. Thromb Haemost 2002; 88: 60-65.
23 Schneiderman J, Rubin E, Nugent DJ. et al. Sequential therapy with activated prothrombin complex concentrates and recombinant FVIIa in patients with severe haemophilia and inhibitors: update of our previous experience. Haemophilia 2007; 13: 244-248.
24 Rosenfeld SB, Watkinson KK, Thompson BH. et al. Pulmonary embolism after sequential use of recombinant factor VIIa and activated prothrombin complex concentrate in a factor VIII inhibitor patient. Thromb Haemost 2002; 87: 925-926.
25 Bui JD, Despotis GD, Trulock EP. et al. Fatal thrombosis after administration of activated prothrombin complex concentrates in a patient supported by extra-corporeal membrane oxygenation who had received activated recombinant factor VII. J Thorac Cardiovasc Surg 2002; 124: 852-854.
26 Knobl P, Derfler K. Extracorporeal immunoadsorption for the treatment of haemophilic patients with inhibitors to factor VIII or IX. Vox Sang 1999; 77 (Suppl. 01) 57-64.
27 Huguet HC, Lasne D, Rothschild C. et al. Extracorporeal adsorption of anti-factor VIII allo-antibodies on randomly functionalized polystyrene resins. Thromb Haemost 2004; 91: 259-266.
28 Zeitler H, Ulrich-Merzenich G, Hess L. et al. Treatment of acquired hemophilia by the Bonn-Malmo Protocol: documentation of an in vivo immunomodulating concept. Blood 2005; 105: 2287-2293.
29 Knobl P, Derfler K, Korninger L. et al. Elimination of acquired factor VIII antibodies by extracorporal antibody-based immunoadsorption (Ig-Therasorb). Thromb Haemost 1995; 74: 1035-1038.
30 Rivard GE, St Louis J, Lacroix S. et al. Immunoadsorption for coagulation factor inhibitors: a retrospective critical appraisal of 10 consecutive cases from a single institution. Haemophilia 2003; 9: 711-716.
31 Watt RM, Bunitsky K, Faulkner EB. et al. Treatment of congenital and acquired hemophilia patients by extracorporeal removal of antibodies to coagulation factors: a review of US clinical studies 1987–1990. Hemophilia Study Group. Transfus Sci 1992; 13: 233-253.
32 Jansen M, Schmaldienst S, Banyai S. et al. Treatment of coagulation inhibitors with extracorporeal immunoadsorption (Ig-Therasorb). Br J Haematol 2001; 112: 91-97.
33 Gjorstrup P, Berntorp E, Larsson L. et al. Kinetic aspects of the removal of IgG and inhibitors in hemophiliacs using protein A immunoadsorption. Vox Sang 1991; 61: 244-250.
34 Saint-Remy JM. Hemophilia factor VIII therapy. Band T-cell tolerance: from basic concepts to clinical practice. Haematologica 2000; 85: 93-96.
35 Brackmann HH, Schwaab R, Effenberger W. et al. Antibodies to factor VIII in hemophilia A patients. Vox Sang 2000; 78 (Suppl. 02) 187-190.
36 Onitilo AA, Skorupa A, Lal A. et al. Rituximab in the treatment of acquired factor VIII inhibitors. Thromb Haemost 2006; 96: 84-87.
37 Grillo-Lopez AJ, Hedrick E, Rashford M. et al. Rituximab: ongoing and future clinical development. Semin Oncol 2002; 29: 105-112.
38 Cartron G, Watier H, Golay J. et al. From the bench to the bedside: ways to improve rituximab efficacy. Blood 2004; 104: 2635-2642.
39 Fischer KG, Deschler B, Lubbert M. Acquired high-titer factor VIII inhibitor: fatal bleeding despite multimodal treatment including rituximab preceded by multiple plasmaphereses. Blood 2003; 101: 3753-3754.
40 Stasi R, Brunetti M, Stipa E. et al. Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia. Blood 2004; 103: 4424-4428.