Acquired hemophilia A with intramuscular hematoma at an unusual age: a case report
https://doi.org/10.21682/2311-1267-2022-9-3-90-92
Abstract
Background. Acquired factor VIII (FVIII) deficiency or acquired hemophilia A (AHA) is very uncommon in children. Patients with AHA usually present with abnormal or unexpected bleeding which may be life-threatening. These patients usually have unexplained, prolonged, and isolated activated partial thromboplastin time (aPTT). Consequently, FVIII activity should be immediately evaluated. Bleeding prevention is important in patients with AHA.
Case report. We present a case of a previously healthy 13-year-old female who presented with intramuscular hematoma, soft tissue hemorrhage, and epistaxis who was eventually diagnosed with AHA.
Conclusion. To our knowledge, the present report is one of the few reported cases of an Asian patient that was diagnosed with acquired hemophilia A at a young age.
Supporting agencies: We would also like to thank Tehran University of Medical Sciences (Tehran, Iran) for their financial support.
About the Authors
S.M.K. Nourbakhsh
Tehran University of Medical Sciences
Islamic Republic of Iran
Islamic Republic of Iran
Seyed Mohammad Kazem Nourbakhsh: MD, Associate Professor of Pediatric Hematology and Oncology, Department of Pediatric Hematology and Oncology, Imam Khomeini Hospital Complex
Keshavarz Blvd., Tehran, 14197-33141
M. Bahadoram
Health Research Institute, Ahvaz Jundishapur University of Medical Sciences
Islamic Republic of Iran
Islamic Republic of Iran
Bahadoram Mohammad: MD, Researcher, Thalassemia and Hemoglobinopathy Research Center
Golestan St., Ahvaz, 15794–61357
S. Eskandarion
Tehran University of Medical Sciences
Islamic Republic of Iran
Islamic Republic of Iran
Sahar Eskandarion: MD, Pediatrician, Department of Pediatric Hematology and Oncology, Imam Khomeini Hospital Complex
Keshavarz Blvd., Tehran, 14197-33141
S. Hassanzadeh
Health Research Institute, Ahvaz Jundishapur University of Medical Sciences
Islamic Republic of Iran
Islamic Republic of Iran
Shakiba Hassanzadeh: MD, Researcher, Thalassemia and Hemoglobinopathy Research Center
Golestan St., Ahvaz, 15794–61357
References
1. Collins P.W., Percy C.L. Advances in the understanding of acquired haemophilia A: implications for clinical practice. Br J Haematol. 2010;148(2):183–94. doi: 10.1111/j.1365-2141.2009.07915.x.
2. Mo L., Bao G.C. Acquired factor VIII deficiency: two case reports and a review of literature. Exp Hematol Oncol. 2017;6:8. doi: 10.1186/s40164-017-0068-3.
3. Lee A.N., Werth V.P. Activation of autoimmunity following use of immunostimulatory herbal supplements. Arch Dermatol. 2004;140(6):723–7. doi: 10.1001/archderm.140.6.723.
4. Vintimilla M., Joseph A., Ranganathan P. Acquired factor VIII inhibitor in Sjögren’s syndrome. Arthritis Care Res (Hoboken). 2010;62(7):1047–50. doi: 10.1002/acr.20147.
5. Boggio L.N., Green D. Acquired hemophilia. Rev Clin Exp Hematol. 2001;5(4):389–404; quiz following 431. doi: 10.1046/j.1468-0734.2001.00049.x.
6. Collins P.W., Hirsch S., Baglin T.P., Dolan G., Hanley J., Makris M., Keeling D.M., Liesner R., Brown S.A., Hay C.R.M., 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(5):1870–7. doi: 10.1182/blood-2006-06-029850.
7. Franchini M., Zaffanello M., Lippi G. Acquired hemophilia in pediatrics: a systematic review. Pediatr Blood Cancer. 2010;55(4):606–11. doi: 10.1002/pbc.22657.
8. Moraca R.J., Ragni M.V. Acquired anti-FVIII inhibitors in children. Haemophilia. 2002;8(1):28–32. doi: 10.1046/j.1365-2516.2002.00574.x.
9. Green D., Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost. 1981;45(3):200–3. PMID: 6792737.
10. Chai-Adisaksopha C., Rattarittamrong E., Norasetthada L., Tantiworawit A., Nawarawong W. Younger age at presentation of acquired haemophilia A in Asian countries: a single-centre study and systematic review. Haemophilia. 2014;20(3):e205–10. doi: 10.1111/hae.12383.
11. Tiede A., Scharf R.E., Dobbelstein C., Werwitzke S. Management of acquired haemophilia A. Hamostaseologie. 2015;35(4):311–8. doi: 10.5482/HAMO-14-11-0064.
12. Tiede A., Werwitzke S., Scharf R.E. Laboratory diagnosis of acquired hemophilia A: limitations, consequences, and challenges. Semin Thromb Hemost. 2014;40(7):803–11. doi: 10.1055/s-0034-1390004.
13. Franchini M., Targher G., Manzato F., Lippi G. Acquired factor VIII inhibitors in oncohematology: a systematic review. Crit Rev Oncol Hematol. 2008;66(3):194–9. doi: 10.1016/j.critrevonc.2007.12.004.
14. Franchini M., Girelli D., Olivieri O., Bozzini C., Guiotto M., Zardini G., Lippi G., Manzato F., Gandin G. Clinical heterogeneity of acquired hemophilia A: a description of 4 cases. Haematologica. 2005;90(3):16. PMID: 15753057.
15. Gandini G., Franchini M., Manzato F., Lippi G., Aprili G. A combination of prednisone, high-dose intravenous immunoglobulin and desmopressin in the treatment of acquired hemophilia A with high-titer inhibitor. Haematologica. 1999;84(11):1054. PMID: 10553173.
16. Baudo F., Collins P., Huth-Kühne A., Lévesque H., Marco P., Nemes L., Pellegrini F., Tengborn L., Knoebl P., EACH2 registry contributors. 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.
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For citations:
Nourbakhsh S., Bahadoram M., Eskandarion S., Hassanzadeh S. Acquired hemophilia A with intramuscular hematoma at an unusual age: a case report. Russian Journal of Pediatric Hematology and Oncology. 2022;9(3):90-92. https://doi.org/10.21682/2311-1267-2022-9-3-90-92
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