Acquired hemophilia
Report of four cases
DOI:
https://doi.org/10.31053/1853.0605.v79.n1.34045Keywords:
hemophilia a, factor viii, autoantibodies, hematomaAbstract
Introduction: Acquired hemophilia is a hemostasis disorder that occurs due to the presence of inhibitory autoantibodies that are directed against coagulation factor VIII. Clinically, it is manifested by spontaneous bleeding mainly in the skin and soft tissues, and unlike hereditary hemophilia ,the presence of hemarthrosis is infrequent. Although many cases are idiopathic, secondary causes must be sought since their treatment is key in the prognosis of the disease. Among these, the presence of autoimmune diseases, neoplasms, drugs, pregnancy, and postpartum stand out. Treatment is based on hemostatic measures to control the bleeding, and therapies to erradicate the autoantibody.
Methodology: In the following manuscript we describe four patients with acquired hemophilia its etiology, treatment, and prognosis.
Results: All four patients had resolution of the bleeding after specific treatment.
Conclusion: Acquired hemophilia is a rare disorder of hemostasis that should be suspected in patients with extensive spontaneous hematomas without prior coagulopathy. Although in many cases an underlying etiology is not found, secondary causes must be sought since their treatment is key to the patient's evolution.
Downloads
References
- Ma AD, Carrizosa D. Acquired Factor VIII Inhibitors: Pathophysiology and Treatment. Hematology Am Soc Hematol Educ Program. 2006;2006(1):432–437. doi: https://doi.org/10.1182/asheducation-2006.1.432.
- Kruse-Jarres R, Kempton CL, Baudo F, Collins PW, Knoebl P, Leissinger CA, et al. Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 2017;92(7):695-705. https://doi.org/10.1002/ajh.24777.
- Collins PW, Hirsch S, Baglin TP, Dolan G, Hanley J, Makris M, et al. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation. Blood. 2007;109(5):1870-1877. doi:10.1182/blood-2006-06-029850.
- Ceresetto JM, Duboscq C, Fondevila C, Tezanos Pinto M. HEMOFILIA ADQUIRIDA (INHIBIDOR ADQUIRIDO DEL FACTOR VIII). Medicina (B. Aires). 2015;75(4):231-238.
- Knoebl P, Marco P, Baudo F, Collins P, Huth-Kühne A, Nemes L, et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012;10(4):622-631. doi:10.1111/j.1538-7836.2012.04654.x.
- Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost. 1981;45(3):200-203.
- Alzate MA, Meschengieser S, Blanco A, Grosso S, Lazzari MA, Sánchez-Luceros A. Acquired Hemophilia A. Experience Of a Single Center. Blood. 2013;122(21):4781. doi: https://doi.org/10.1182/blood.V122.21.4781.4781.
- Villalba NL, Zulfiqar AA, Alonso Ortiz MB, Jannot X, Syrovatkova A, Andres E. Hemofilia adquirida como manifestación inicial de recidiva de adenocarcinoma colorrectal. Medicina (B Aires). 2021;81(2):286-288.
- Franchini M, Glingani C, De Donno G, Casari S, Caruso B, Terenziani I, et al. The first case of acquired hemophilia A associated with SARS-CoV-2 infection. Am J Hematol. 2020;95(8):E197-E198. doi: 10.1002/ajh.25865.
- Delgado J, Jimenez-Yuste V, Hernandez-Navarro F, Villar A. Acquired haemophilia: review and meta-analysis focused on therapy and prognostic factors. Br J Haematol. 2003;121(1):21-35. doi:10.1046/j.1365-2141.2003.04162.x.
- Collins P, Baudo F, Knoebl P, Lévesque H, Nemes L, Pellegrini F, 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.
- Quarchioni M. Actualización en el abordaje terapéutico del inhibidor adquirido de FVIII (Hemofilia adquirida).Revista Hematología. 2017;21(1):49-58.
- Girardi D, Silva D, Villaça P, Souza C, Fonseca L, Bastos D, et al. Acquired hemophilia A in a patient with advanced prostate cancer. Autops Case Rep. 2015;5(2):55-59. doi:10.4322/acr.2015.008
- Kleinman M. Anti-inhibitor coagulant complex for the rescue therapy of acquired inhibitors to factor VIII: case report and review of the literature. Haemophilia. 2002;8(5):694-7. doi:10.1046/j.1365-2516.2002.00652.x.
- Baudo F, Collins P, Huth-Kühne A, Lévesque H, Pascual M, László N, et al. Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry. Blood. 2012;120(1):39-46. doi:10.1182/blood-2012-02-408930.
Published
How to Cite
Issue
Section
License
Copyright (c) 2022 Universidad Nacional de Córdoba
![Creative Commons License](http://i.creativecommons.org/l/by-nc/4.0/88x31.png)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
The Faculty of Medical Sciences Journal (RFCM) subscribes to the Open Access policy and does not charge authors fees for publishing, nor does it charge readers fees for accessing published articles (APC).