获得性血友病A免疫抑制剂的治疗进展_West China Medical Journal

Authors：

 魏卿 ,  牛挺

四川大学华西医院血液内科（成都，610041）;

Corresponding author：

牛挺, Email: tingniu@sina.com

Keywords：

获得性血友病A; 凝血因子Ⅷ抑制物; 免疫抑制治疗

DOI：

10.7507/1002-0179.20140196

Video：

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获得性血友病A是一种发病率极低但病死率较高的自身免疫性疾病。多种原因导致患者体内产生了特异结合、灭活凝血因子Ⅷ的凝血因子抑制物而发病。患者在其体内凝血因子抑制物完全清除之前都处于严重出血甚至致死的风险之中。因此，如何正确合理地选择免疫抑制剂来进行抑制物清除治疗是消除患者出血风险，达到长期稳定完全缓解以及最终治愈该病的重要环节。

Citation： 魏卿, 牛挺. 获得性血友病A免疫抑制剂的治疗进展. West China Medical Journal, 2014, 29(4): 646-650. doi: 10.7507/1002-0179.20140196 Copy

1.	Kaushansky LB, Kipps SP. 威廉姆斯血液病学[M]. 陈竺,陈赛娟,译. 8版. 北京:人民卫生出版社, 2011:1941-1944.
2.	张磊, 杨仁池. 关于获得性血友病A诊断与治疗国际建议的解读[J]. 国际输血及血液学杂志, 2010, 33(2):172-175.
3.	Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, et al. Acquired haemophilia:review and meta-analysis focused on therapy and prognostic factors[J]. Br J Haematol, 2003, 121(1):21-35.
4.	Shetty S, Bhave M, Ghosh K. Acquired hemophilia A:diagnosis, aetiology, clinical spectrum and treatment options[J]. Autoimmun Rev, 2011, 10(6):311-316.
5.	Franchini M. Acquired hemophilia A[J]. Hematology, 2006, 11(2):119-125.
6.	Toschi V, Baudo F. Diagnosis, laboratory aspects and management of acquired hemophilia A[J]. Intern Emerg Med, 2010, 5(4):325-333.
7.	Collins P, Baudo F, Knoebl P, et al. Immunosuppression for acquired hemophilia A:results from the European Acquired Haemophilia Registry (EACH2)[J]. Blood, 2012, 120(1):47-55.
8.	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[J]. Blood, 2007, 109(5):1870-1877.
9.	Franchini M, Targher G, Manzato F, et al. Acquired factorⅧ inhibitors in oncohematology:a systematic review[J]. Crit Rev Oncol Hematol, 2008, 66(3):194-199.
10.	Aouba A, Rey G, Pavillon G, et al. Deaths associated with acquired haemophilia in France from 2000 to 2009:multiple cause analysis for best care strategies[J]. Haemophilia, 2012, 18(3):339-344.
11.	Kruse-Jarres R, Fang J, Leissinger CA, et al. Reduced production of IFN-γ and LT-α is associated with successful prednisone therapy in patients with acquired hemophilia A:a pilot study[J]. Thromb Res, 2011, 128(5):e86-e90.
12.	Tengborn L, Baudo F, Huth-Kühne A, et al. Pregnancy-associated acquired haemophilia A:results from the European Acquired Haemophilia (EACH2) registry[J]. BJOG, 2012, 119(12):1529-1537.
13.	Collins PW. Therapeutic challenges in acquired factorⅧ deficiency[J]. Hematology Am Soc Hematol Educ Program, 2012:369-374.
14.	Tiede A, Huth-Kühne A, Oldenburg J, et al. Immunosuppressive treatment for acquired haemophilia:current practice and future directions in Germany, Austria and Switzerland[J]. Ann Hematol, 2009, 88(4):365-370.
15.	Huang SY, Tsay W, Lin SY, et al. A study of 65 patients with acquired hemophilia A in Taiwan[J]. J Formos Med Assoc, 2013(4):1-7.
16.	Reeves BN, Key NS. Acquired hemophilia in malignancy[J]. Thromb Res, 2012, 129(Suppl 1):S66-S68.
17.	Sallah S, Wan JY. Inhibitors against factorⅧ associated with the use of interferon-alpha and fludarabine[J]. Thromb Haemost, 2001, 86(4):1119-1121.
18.	Kruse-Jarres R, Fang J, Leissinger CA, et al. Rituximab therapy modulates IFN-γ and IL-4 gene expression in a patient with acquired haemophilia A[J]. Br J Haematol, 2010, 148(1):176-178.
19.	Huang SY, Tsay W, Lin HY, et al. A study of 46 patients with acquired haemophilia A in Taiwan finds elevated LDH level and mucosal bleedings associated with reduced response to immunosuppressive therapy[J]. Haemophilia, 2012, 18(3):e71-e72.
20.	Adrian W, Hearn JC, Adam S, et al. Rituximab in the treatment of acquired factor Ⅷ inhibitors[J]. Blood, 2002, 100(9):3426-3428.
21.	Castaman G, Tosetto A. Is a reduced intensity treatment with Rituximab effective in acquired haemophilia A?[J]. Haemophilia, 2011, 17(5):817-818.
22.	Xu Y, Zhang X, Zhao Y, et al. Successful treatment of a patient with acquired haemophilia A with a combination of a low-dose rituximab and recombinant human FⅦa[J]. Haemophilia, 2013, 19(2):e95-e96.
23.	Franchini M. Rituximab in the treatment of adult acquired hemophilia A:a systematic review[J]. Crit Rev Oncol Hematol, 2007, 63(1):47-52.
24.	Aggarwal A, Grewal R, Green RJ, et al. Rituximab for autoimmune haemophilia:a proposed treatment algorithm[J]. Haemophilia, 2005, 11(1):13-19.
25.	Boles JC, Key NS, Kasthuri R, et al. Single-center experience with rituximab as first-line immunosuppression for acquired hemophilia[J]. J Thromb Haemost, 2011, 9(7):1429-1431.

1. Kaushansky LB, Kipps SP. 威廉姆斯血液病学[M]. 陈竺,陈赛娟,译. 8版. 北京:人民卫生出版社, 2011:1941-1944.
2. 张磊, 杨仁池. 关于获得性血友病A诊断与治疗国际建议的解读[J]. 国际输血及血液学杂志, 2010, 33(2):172-175.
3. Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, et al. Acquired haemophilia:review and meta-analysis focused on therapy and prognostic factors[J]. Br J Haematol, 2003, 121(1):21-35.
4. Shetty S, Bhave M, Ghosh K. Acquired hemophilia A:diagnosis, aetiology, clinical spectrum and treatment options[J]. Autoimmun Rev, 2011, 10(6):311-316.
5. Franchini M. Acquired hemophilia A[J]. Hematology, 2006, 11(2):119-125.
6. Toschi V, Baudo F. Diagnosis, laboratory aspects and management of acquired hemophilia A[J]. Intern Emerg Med, 2010, 5(4):325-333.
7. Collins P, Baudo F, Knoebl P, et al. Immunosuppression for acquired hemophilia A:results from the European Acquired Haemophilia Registry (EACH2)[J]. Blood, 2012, 120(1):47-55.
8. 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[J]. Blood, 2007, 109(5):1870-1877.
9. Franchini M, Targher G, Manzato F, et al. Acquired factorⅧ inhibitors in oncohematology:a systematic review[J]. Crit Rev Oncol Hematol, 2008, 66(3):194-199.
10. Aouba A, Rey G, Pavillon G, et al. Deaths associated with acquired haemophilia in France from 2000 to 2009:multiple cause analysis for best care strategies[J]. Haemophilia, 2012, 18(3):339-344.
11. Kruse-Jarres R, Fang J, Leissinger CA, et al. Reduced production of IFN-γ and LT-α is associated with successful prednisone therapy in patients with acquired hemophilia A:a pilot study[J]. Thromb Res, 2011, 128(5):e86-e90.
12. Tengborn L, Baudo F, Huth-Kühne A, et al. Pregnancy-associated acquired haemophilia A:results from the European Acquired Haemophilia (EACH2) registry[J]. BJOG, 2012, 119(12):1529-1537.
13. Collins PW. Therapeutic challenges in acquired factorⅧ deficiency[J]. Hematology Am Soc Hematol Educ Program, 2012:369-374.
14. Tiede A, Huth-Kühne A, Oldenburg J, et al. Immunosuppressive treatment for acquired haemophilia:current practice and future directions in Germany, Austria and Switzerland[J]. Ann Hematol, 2009, 88(4):365-370.
15. Huang SY, Tsay W, Lin SY, et al. A study of 65 patients with acquired hemophilia A in Taiwan[J]. J Formos Med Assoc, 2013(4):1-7.
16. Reeves BN, Key NS. Acquired hemophilia in malignancy[J]. Thromb Res, 2012, 129(Suppl 1):S66-S68.
17. Sallah S, Wan JY. Inhibitors against factorⅧ associated with the use of interferon-alpha and fludarabine[J]. Thromb Haemost, 2001, 86(4):1119-1121.
18. Kruse-Jarres R, Fang J, Leissinger CA, et al. Rituximab therapy modulates IFN-γ and IL-4 gene expression in a patient with acquired haemophilia A[J]. Br J Haematol, 2010, 148(1):176-178.
19. Huang SY, Tsay W, Lin HY, et al. A study of 46 patients with acquired haemophilia A in Taiwan finds elevated LDH level and mucosal bleedings associated with reduced response to immunosuppressive therapy[J]. Haemophilia, 2012, 18(3):e71-e72.
20. Adrian W, Hearn JC, Adam S, et al. Rituximab in the treatment of acquired factor Ⅷ inhibitors[J]. Blood, 2002, 100(9):3426-3428.
21. Castaman G, Tosetto A. Is a reduced intensity treatment with Rituximab effective in acquired haemophilia A?[J]. Haemophilia, 2011, 17(5):817-818.
22. Xu Y, Zhang X, Zhao Y, et al. Successful treatment of a patient with acquired haemophilia A with a combination of a low-dose rituximab and recombinant human FⅦa[J]. Haemophilia, 2013, 19(2):e95-e96.
23. Franchini M. Rituximab in the treatment of adult acquired hemophilia A:a systematic review[J]. Crit Rev Oncol Hematol, 2007, 63(1):47-52.
24. Aggarwal A, Grewal R, Green RJ, et al. Rituximab for autoimmune haemophilia:a proposed treatment algorithm[J]. Haemophilia, 2005, 11(1):13-19.
25. Boles JC, Key NS, Kasthuri R, et al. Single-center experience with rituximab as first-line immunosuppression for acquired hemophilia[J]. J Thromb Haemost, 2011, 9(7):1429-1431.

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