獲得性血友病A免疫抑制劑的治療進展_West China Medical Journal

Authors：

 魏卿 ,  牛挺

四川大學華西醫院血液內科（成都，610041）;

Corresponding?author：

牛挺, Email: tingniu@sina.com

Keywords：

獲得性血友病A; 凝血因子Ⅷ抑制物; 免疫抑制治療

DOI：

10.7507/1002-0179.20140196

Video：

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獲得性血友病A是一種發病率極低但病死率較高的自身免疫性疾病。多種原因導致患者體內產生了特異結合、滅活凝血因子Ⅷ的凝血因子抑制物而發病。患者在其體內凝血因子抑制物完全清除之前都處于嚴重出血甚至致死的風險之中。因此，如何正確合理地選擇免疫抑制劑來進行抑制物清除治療是消除患者出血風險，達到長期穩定完全緩解以及最終治愈該病的重要環節。

Citation： 魏卿, 牛挺. 獲得性血友病A免疫抑制劑的治療進展. West China Medical Journal, 2014, 29(4): 646-650. doi: 10.7507/1002-0179.20140196 Copy

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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. 威廉姆斯血液病學[M]. 陳竺,陳賽娟,譯. 8版. 北京:人民衛生出版社, 2011:1941-1944.
2. 張磊, 楊仁池. 關于獲得性血友病A診斷與治療國際建議的解讀[J]. 國際輸血及血液學雜志, 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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獲得性血友病A免疫抑制劑的治療進展

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