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Vol. 127, No. 1, 2012
Issue release date: December 2011

Efficacy of Low-Dose Rituximab in a Refractory Acquired Factor VIII Inhibitor Case Secondary to Pemphigus

Gao Y. · Liu S. · Ma G. · Lv L. · Su Y.
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Abstract

Acquired factor VIII (FVIII) inhibitor induces a bleeding disorder caused by specific antibodies to FVIII. The cause of approximately one fifth of cases can be attributed to autoimmune disorders, such as pemphigus. Here, we describe a case of refractory acquired FVIII inhibitor in a patient with primary pemphigus and its successful treatment with low-dose rituximab. Coagulation studies revealed a prolonged activated partial thromboplastin time, which could not be corrected with the mixing test. At the same time, the FVIII activity level was significantly reduced, and the FVIII inhibitor titer was elevated. A treatment regimen with prednisolone/cyclophosphamide followed by prednisolone/cyclosporine was used. The patient temporarily responded but then became resistant to these medicines. However, subsequent treatment with low-dose rituximab achieved considerable clinical and laboratory improvement in the same patient. Follow-up at 6 months revealed a low level of residual FVIII inhibitor activity with normal coagulation functions. No drug-related side effects were detected. In conclusion, our results indicate that low-dose rituximab might be an effective and safe treatment for patients with acquired FVIII inhibitor.



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References

  1. Huth-Kühne A, Baudo F, Collins P, Ingerslev J, Kessler CM, Lévesque H, Castellano ME, Shima M, St-Louis J: International recommendations on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica 2009;94:566–575.
  2. Stasi R, Brunetti M, Stipa E, Amadori S: Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia. Blood 2004;103:4424–4428.
  3. Kelesidis T, Raphael J, Blanchard E, Parameswaran R: Acquired hemophilia as the cause of life-threatening hemorrhage in a 94-year-old man: a case report. J Med Case Reports 2010;4:231.
  4. Elezović I: Acquired haemophilia syndrome: pathophysiology and therapy. Srp Arh Celok Lek 2010;138(suppl 1):64–68.

    External Resources

  5. Ayoola A, Mohsini W, Aung S: Acquired factor VIII inhibitor in a patient with dermatomyositis – a case study. Acta Haematol 2008;119:8–11.
  6. Franchini M, Lippi G: Acquired factor VIII inhibitors. Blood 2008;112:250–255.
  7. Aggarwal A, Grewal R, Green RJ, Boggio L, Green D, Weksler BB, Wiestner A, Schechter GP: Rituximab for autoimmune haemophilia: a proposed treatment algorithm. Haemophilia 2005;11:13–19.
  8. Green D, Lechner K: A survey of 215 non-hemophilic patients with inhibitors to factor VIII. Thromb Haemost 1981;45:200–203.
  9. Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, Keeling DM, Liesner R, Brown SA, Hay CR, UK Haemophilia Centre Doctors’ Organisation: 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.
  10. Boggio LN, Green D: Acquired hemophilia. Rev Clin Exp Hematol 2001;5:389–404.
  11. Collins P, Baudo F, Huth-Kühne A, Ingerslev J, Kessler CM, Castellano ME, Shima M, St-Louis J, Lévesque H: Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Res Notes 2010;3:161.
  12. Shima M: Diagnosis and treatment of acquired hemophilia. Rinsho Ketsueki 2010;51:1531–1538.

    External Resources

  13. Machado P, Raya JM, Martín T, Morabito L, Brito ML, Rodríguez-Martín JM: Successful response to rituximab in two cases of acquired haemophilia refractory to standard-therapy. Int J Hematol 2008;87:545–549.
  14. Halbertsma FJ, van der Linden PW, Mauser-Bunschoten EP: A patient with acquired haemophilia A and pemphigus. Neth J Med 1998;52:190–192.
  15. Onitilo AA, Skorupa A, Lal A, Ronish E, Mercier RJ, Islam R, Lazarchick J: Rituximab in the treatment of acquired factor VIII inhibitors. Thromb Haemost 2006;96:84–87.
  16. Provan D, Butler T, Evangelista ML, Amadori S, Newland AC, Stasi R: Activity and safety profile of low-dose rituximab for the treatment of autoimmune cytopenias in adults. Haematologica 2007;92:1695–1698.
  17. Kimby E: Tolerability and safety of rituximab (MabThera). Cancer Treat Rev 2005;31:456–473.
  18. Covelli M, Sarzi-Puttini P, Atzeni F, Macchioni P: Safety of rituximab in rheumatoid arthritis. Reumatismo 2010;62:101–106.


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