Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review
Tóm tắt
We report the case of a 38-year-old female patient with systemic lupus erythematosus (SLE) who developed acquired hemophilia caused by factor VIII (FVIII) inhibitors. She manifested spontaneous bleeding symptoms such as ecchymoses and hematuria. Laboratory findings showed an isolated prolongation of the activated partial thromboplastin time, reduced FVIII activity, and a high titer of FVIII inhibitors. She was successfully treated with oral predonisolone and cyclosporine in combination with steroid and cyclophosphamide pulse therapy.
Tài liệu tham khảo
Franchini M, Gandini G, Di Paolantonio T, Mariani G. Acquired hemophilia A: a concise review. Am J Hematol. 2005;80:55–63.
Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost. 1981;45:200–3.
Ma AD, Carrizosa D. Acquired factor VIII inhibitors: pathophysiology and treatment. Hematology Am Soc Hematol Educ Program. 2006;432–7.
Pirner K, Rosler W, Kalden JR, Manger B. Long-term remission after i.v. immunoglobulin therapy in acquired antihemophilic factor hemophilia with systemic lupus erythematosus. Z Rheumatol. 1990;49:378–81.
Schwartz RS, Gabriel DA, Aledort LM, Green D, Kessler CM. A prospective study of treatment of acquired (autoimmune) factor VIII inhibitors with high-dose intravenous gammaglobulin. Blood. 1995;86:797–804.
Schulman S, Langevitz P, Livneh A, Mortinowitz U, Seligsohn U, Varon D. Cyclosporine therapy for acquired factor VIII inhibitor in a patient with systemic lupus erythematosus. Thromb Haemost. 1996;76:344–6.
Lafferty TE, Smith JB, Schuster SJ, DeHoratius RJ. Treatment of acquired factor VIII inhibitor using intravenous immunoglobulin in two patients with systemic lupus erythematosus. Arthritis Rheum. 1997;40:775–8.
Trotta F, Bajocchi G, La Corte R, Moratelli S, Sun LY. Long-lasting remission and successful treatment of acquired factor VIII inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus. Rheumatology (Oxford). 1999;38:1007–9.
Ishikawa T, Tsukamoto N, Suto M, Uchiumi H, Mitsuhashi H, Yokohama A, et al. Acquired hemophilia A in a patient with systemic lupus erythematosus. Intern Med. 2001;40:541–3.
Nishino Y, Ueki K, Suto M, Uchiumi H, Ota F, Tamura S, et al. Successful treatment of patients with rheumatic disorders and acquired factor VIII inhibitors with cyclophosphamide and prednisolone combination therapy: two case reports. J Int Med Res. 2001;29:432–6.
Kornfeld S, Veyssier-Belot C, Vinceneux A, Renier JL, Du-Boutin le TH, Pauwels C. Acquired haemophilia in a patient with systemic lupus erythematosus. Ann Dermatol Venereol. 2002;129:316–9.
Onishi S, Hojo N, Sakai I, Yasukawa M, Hato T, Minamoto Y, et al. Rupture of the gallbladder in a patient with acquired factor VIII inhibitors and systemic lupus erythematosus. Intern Med. 2004;43:1073–7.
Green D, Rademaker AW, Briet E. A prospective, randomized trial of prednisone and cyclophosphamide in the treatment of patients with factor VIII autoantibodies. Thromb Haemost. 1993;70:753–7.
Sultan Y, Kazatchkine MD, Maisonneuve P, Nydegger UE. Anti-idiotypic suppression of autoantibodies to factor VIII (antihaemophilic factor) by high-dose intravenous gammaglobulin. Lancet. 1984;2:765–8.
Francesconi M, Korninger C, Thaler E, Niessner H, Hocker P, Lechner K. Plasmapheresis: its value in the management of patients with antibodies to factor VIII. Haemostasis. 1982;11:79–86.
Freedman J, Garvey MB. Immunoadsorption of factor VIII inhibitors. Curr Opin Hematol. 2004;11:327–33.
Zeitler H, Ulrich-Merzenich G, Hess L, Konsek E, Unkrig C, Walger P, et al. Treatment of acquired hemophilia by the Bonn-Malmo Protocol: documentation of an in vivo immunomodulating concept. Blood. 2005;105:2287–93.
Spero JA, Lewis JH, Hasiba U. Corticosteroid therapy for acquired F VIII:C inhibitors. Br J Haematol. 1981;48:635–42.
Herbst KD, Rapaport SI, Kenoyer DG, Stanton W, Feinstein DI. Syndrome of an acquired inhibitor of factor VIII responsive to cyclophosphamide and prednisone. Ann Intern Med. 1981;95:575–8.
Kain S, Copeland TS, Leahy MF. Treatment of refractory autoimmune (acquired) haemophilia with anti-CD20 (rituximab). Br J Haematol. 2002;119:578.
Wiestner A, Cho HJ, Asch AS, Michelis MA, Zeller JA, Peerschke EI, et al. Rituximab in the treatment of acquired factor VIII inhibitors. Blood. 2002;100:3426–8.
Stasi R, Brunetti M, Stipa E, Amadori S. Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia. Blood. 2004;103:4424–8.
Hart HC, Kraaijenhagen RJ, Kerckhaert JA, Verdel G, Freen M, van de Wiel A. A patient with a spontaneous factor VIII:C autoantibody: successful treatment with cyclosporine. Transplant Proc. 1988;20:323–8.
Pfliegler G, Boda Z, Harsfalvi J, Flora-Nagy M, Sari B, Pecze K, et al. Cyclosporin treatment of a woman with acquired haemophilia due to factor VIII:C inhibitor. Postgrad Med J. 1989;65:400–2.
Brox AG, Laryea H, Pelletier M. Successful treatment of acquired factor VIII inhibitors with cyclosporin. Am J Hematol. 1998;57:87–8.
Petrovic M, Derom E, Baele G. Cyclosporine treatment of acquired hemophilia due to factor VIII antibodies. Haematologica. 2000;85:895–6.
Au WY, Lam CC, Kwong YL. Successful treatment of acquired factor VIII inhibitor with cyclosporin. Haemophilia. 2004;10:98–100.
Maize JC Jr, Cohen JB. Cyclosporine controls epidermolysis bullosa acquisita co-occurring with acquired factor VIII deficiency. Int J Dermatol. 2005;44:692–4.
Pardos-Gea J, Ordi-Ros J, Altisent C, Balada E, Perez-Lopez J, Vilardell M. Acquired haemophilia A: successful treatment with immunosuppression, methylprednisolone pulses and oral cyclosporin. Thromb Haemost. 2006;95:735–7.
Thatayatikom A, White AJ. Rituximab: a promising therapy in systemic lupus erythematosus. Autoimmun Rev. 2006;5:18–24.
Eisenberg R, Albert D. B-cell targeted therapies in rheumatoid arthritis and systemic lupus erythematosus. Nat Clin Pract Rheumatol. 2006;2:20–7.
Aggarwal A, Grewal R, Green RJ, Boggio L, Green D, Weksler BB, et al. Rituximab for autoimmune haemophilia: a proposed treatment algorithm. Haemophilia. 2005;11:13–9.