2021, Number 4
<< Back
Rev Hematol Mex 2021; 22 (4)
Acquired hemophilia A secondary to Epstein-Barr virus infection
Peralta-Amaro AL, Vázquez-Hernández A, Hernández-Martínez C, Oliva-Pérez EA, Olvera-Acevedo A, Vera-Lastra OL, García-Chávez J, Lucas-Hernández A
Language: English
References: 11
Page: 266-270
PDF size: 245.61 Kb.
ABSTRACT
Background: Acquired hemophilia A is a bleeding disorder that is usually presented
as an idiopathic entity; nevertheless, it has been attributed to other factors,
such as autoimmune, malignancy, infections, among others. The diagnosis is made
when autoantibodies against epitopes of factor VIII (FVIII) are evidenced, causing the
neutralization of the FVIII coagulant activity.
Clinical cases: A 34-year-old female patient that presented with coagulopathy after
Epstein-Barr virus (EBV) infection verified by IgG against the nuclear antigen of EBV,
secondary causes, like autoimmune, pregnancy, drugs, and others were ruled out. This
patient improved after receiving treatment with factor VIII, and complete remission was
obtained with rituximab and steroids. After a three-year follow up there has not been
any new episode of coagulopathy.
Conclusions: Acquired hemophilia A is a life-threatening entity, if not diagnosed
and treated promptly it can be fatal. To the best of our knowledge, this is the first case
of Epstein-Barr virus-associated acquired hemophilia A.
REFERENCES
Mazzucconi MG, Baldacci E, Ferretti A, Santoro C. Acquiredhaemophilia A: An intriguing disease. Mediterr JHematol Infect Dis 2020; 12 (1): e2020045. doi: 10.4084/MJHID.2020.045.
Franchini M, Vaglio S, Marano G, Mengoli C, Gentili S,Pupella S, et al. Acquired hemophilia A: a review of recentdata and new therapeutic options. Hematology 2017; 22(9): 514-20. doi: 10.1080/10245332.2017.1319115.
W Collins P, Chalmers E, Hart D, Jennings I, Liesner R, RangarajanS, et al. Diagnosis and management of acquiredcoagulation inhibitors: a guideline from UKHCDO. Br JHaematol 2013; 162 (6): 758-73. doi: 10.1111/bjh.12463.
García-Chávez J, Majluf-Cruz A. Acquired hemophilia.Gac Med Mex 2020; 156 (1): 67-77. doi: 10.24875/GMM.19005469.
Kruse-Jarres R, Kempton CL, Baudo F, Collins PW, Knoebl P,Leissinger CA, et al. Acquired hemophilia A: Updated reviewof evidence and treatment guidance. Am J Hematol 2017;92 (7): 695-705. doi: 10.1002/ajh.24777.
Tiede A, Klamroth R, Scharf RE, Trappe RU, Holstein K,Huth-Kühne A, et al. Prognostic factors for remission ofand survival in acquired hemophilia A (AHA): results fromthe GTH-AH 01/2010 study. Blood 2015; 125 (7): 1091-97.doi: 10.1182/blood-2014-07-587089.
Sborov DW, Rodgers GM. How I manage patients withacquired haemophilia A. Br J Haematol 2013; 161 (2):157-65. doi: 10.1111/bjh.12228.
Aggarwal A, Grewal R, Green RJ, Boggio L, Green D, WekslerBB, et al. Rituximab for autoimmune haemophilia: aproposed treatment algorithm. Haemophilia 2005; 11 (1):13-19. doi: 10.1111/j.1365-2516.2005.01060.x.
Astermark J, Donfield SM, DiMichele DM, Gringeri A, GilbertSA, Waters J, et al. A randomized comparison of bypassingagents in hemophilia complicated by an inhibitor: theFEIBA NovoSeven Comparative (FENOC) Study. Blood 2007;
109 (2): 546-51. doi: 10.1182/blood-2006-04-017988.10. Collins P, Baudo F, Knoebl P, Lévesque H, Nemes L, PellegriniF, et al. Immunosuppression for acquired hemophilia A:results from the European Acquired Haemophilia Registry(EACH2). Blood 2012; 120 (1): 47-55. doi:10.1182/blood-2012-02-409185.
Loloi J, Moroi MK, Songdej N, Al-Mondhiry HA. Long-termoutcomes in the treatment of acquired hemophilia A: a16-year single institution prospective cohort experience.Blood Coagul Fibrinolysis. 2020;31(1):43-47. doi: 10.1097/MBC.0000000000000874.
Full text