Acquired hemophilia A presenting as progressive intra‑abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review

Xu,Liying; Chen,Junfa; Zhou,Xiajuan; Wu,Lijuan; Tong,Yixin; Zhu,Ni; Huang,Xiaomin; Zhang,Zhuoyi

doi:10.3892/etm.2018.7031

January-2019 Volume 17 Issue 1

Full Size Image

Journals

International Journal of Molecular Medicine

International Journal of Molecular Medicine is an international journal devoted to molecular mechanisms of human disease.

International Journal of Oncology

International Journal of Oncology is an international journal devoted to oncology research and cancer treatment.

Molecular Medicine Reports

Covers molecular medicine topics such as pharmacology, pathology, genetics, neuroscience, infectious diseases, molecular cardiology, and molecular surgery.

Oncology Reports

Oncology Reports is an international journal devoted to fundamental and applied research in Oncology.

Experimental and Therapeutic Medicine

Experimental and Therapeutic Medicine is an international journal devoted to laboratory and clinical medicine.

Oncology Letters

Oncology Letters is an international journal devoted to Experimental and Clinical Oncology.

Biomedical Reports

Explores a wide range of biological and medical fields, including pharmacology, genetics, microbiology, neuroscience, and molecular cardiology.

Molecular and Clinical Oncology

International journal addressing all aspects of oncology research, from tumorigenesis and oncogenes to chemotherapy and metastasis.

World Academy of Sciences Journal

Multidisciplinary open-access journal spanning biochemistry, genetics, neuroscience, environmental health, and synthetic biology.

International Journal of Functional Nutrition

Open-access journal combining biochemistry, pharmacology, immunology, and genetics to advance health through functional nutrition.

International Journal of Epigenetics

Publishes open-access research on using epigenetics to advance understanding and treatment of human disease.

Medicine International

An International Open Access Journal Devoted to General Medicine.

January-2019 Volume 17 Issue 1

Full Size Image

Case Report Open Access

Acquired hemophilia A presenting as progressive intra‑abdominal hemorrhage, muscle hemorrhage and hemothorax postpartum: A case report and literature review

Authors:
- Liying Xu
- Junfa Chen
- Xiajuan Zhou
- Lijuan Wu
- Yixin Tong
- Ni Zhu
- Xiaomin Huang
- Zhuoyi Zhang
View Affiliations / Copyright

Affiliations: Department of Emergency, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang 310006, P.R. China, Department of Hematology, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang 310006, P.R. China, Department of General Practice, The First Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, Zhejiang 310006, P.R. China

Copyright: © Xu et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
Pages: 633-638
|
Published online on: November 29, 2018

https://doi.org/10.3892/etm.2018.7031
Expand metrics +

Abstract

Acquired hemophilia A (AHA) is a rare antibody‑mediated condition in which autoantibodies form against a coagulation factor, most commonly factor VIII (FVIII), causing severe coagulopathy. Here the present report presents a case of AHA in a 35‑year‑old postpartum woman with continuous polyserous bloody effusions who was admitted to the First Affiliated Hospital of Zhejiang Chinese Medical University (Hangzhou, China) in October 2017 without a history of trauma, anticoagulation treatment or coagulopathy. At presentation, the patient's hemoglobin level was low (70 g/l; normal range: 115‑150 g/l) g/l, blood pressure was 89/58 mmHg (normal range, 90‑140/60‑90 mmHg), and activated partial thromboplastin time was 68.4 sec (normal range: 25.0‑36.0 sec), with a normal international normalized ratio (0.94; normal range, 0.8‑1.2). The reaction time in thrombography was prolonged (35.8 min; normal range: 5‑10 min), coagulation FVIII had markedly decreased activity (12.6%; normal range, 60‑150%), and FVIII inhibitor had a high titer [7.4 Bethesda units (BU)/ml; normal range, 0‑0.6 BU/ml]. Notably, the patient's autoantibody level was markedly higher than normal (1:320; normal range: <1:100). The patient was successfully treated with bleeding control, eradication of FVIII inhibitor, and treatment of the underlying disease. To the best of our knowledge, this is the first case of AHA with polyserous bloody effusions in a patient with an autoimmune disorder during the postpartum period. Reports of such rare cases will aid the characterization of disease pathogenesis, which may in turn lead to the recognition and timely treatment of this rare disorder.

View Figures

View References

1	Vautier M, de Boysson H, Creveuil C, Repesse Y, Borel-Derlon A, Troussard X, Damaj GL, Bienvenu B, Gautier P and Aouba A: Influence of factor VIII level and its inhibitor titer on the therapeutic response to corticosteroids alone in the management of acquired hemophilia. Medicine (Baltimore). 95:e52322016. View Article : Google Scholar : PubMed/NCBI
2	Sakurai Y and Takeda T: Acquired hemophilia A: A frequently overlooked autoimmune hemorrhagic disorder. J Immunol Res. 2014:3206742014. View Article : Google Scholar : PubMed/NCBI
3	Collins P, Baudo F, Huth-Kühne A, Ingerslev J, Kessler CM, Castellano MEM, Shima M, St-Louis J and Lévesque H: Research article Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Research Notes Notes Research Notes. 3(161)2010.
4	Aljasser MI, Sladden C, Crawford RI and Au S: Bullous pemphigoid associated with acquired hemophilia A: A rare association of autoimmune disease. J Cutan Med Surg. 18:123–126. 2014. View Article : Google Scholar : PubMed/NCBI
5	Olteanu M, Niţu M and Golli A: Tuberculosis mesenteric adenopathy and polyserositis. Rom J Morphol Embryol. 53:835–840. 2012.PubMed/NCBI
6	Kruse-Jarres R, Kempton CL, Baudo F, Collins PW, Knoebl P, Leissinger CA, Tiede A and Kessler CM: Acquired hemophilia A: Updated review of evidence and treatment guidance. Am J Hematol. 92:695–705. 2017. View Article : Google Scholar : PubMed/NCBI
7	Bitting RL, Bent S, Li Y and Kohlwes J: The prognosis and treatment of acquired hemophilia: A systematic review and meta-analysis. Blood Coagul Fibrinolysis. 20:517–523. 2009. View Article : Google Scholar : PubMed/NCBI
8	Chinese society of hematology: Chinese expert consensus on the diagnosis and treatment of acquired hemophilia A. Zhonghua Xue Ye Xue Za Zhi. 35:575–576. 2014.(In Chinese). PubMed/NCBI
9	Coppola A, Franchini M, Castaman G, Santagostino E, Santoro C, Santoro RC, Morfini M, Minno GD and Rocino A; and on behalf of the AICE ad hoc Working Group, : Treatment regimens with bypassing agents in patients with hemophilia A and inhibitors: A survey from the Italian association of hemophilia centers (AICE). Semin Thromb Hemost. 44:551–560. 2018. View Article : Google Scholar : PubMed/NCBI
10	Brandt JT, Barna LK and Triplett DA: Laboratory identification of lupus anticoagulants: Results of the second international workshop for identification of lupus anticoagulants. Thromb Haemost. 74:1597–1603. 1995. View Article : Google Scholar : PubMed/NCBI
11	Kumano O, Amiral J, Dunois C, Peyrafitte M and Moore GW: Paired APTTs of low and high lupus anticoagulant sensitivity permit distinction from other abnormalities and achieve good lupus anticoagulant detection rates in conjunction with dRVVT. Int J Lab Hematol. 24:2018.
12	Thrombosis and Hemostasis Group, Chinese Society of Hematology, Chinese Medical Association: Chinese expert consensus on the diagnosis and treatment of acquired hemophilia A (Chinese). Chin J Hematol. 35:575–576. 2014.
13	Greaves M, Cohen H, MacHin SJ and Mackie I: Guidelines on the investigation and management of the antiphospholipid syndrome. BrJ Haematol. 109:704–715. 2000. View Article : Google Scholar
14	Theodossiades G, Tsevrenis V, Nomikou E, Dadiotis L and Kontopoulou-Griva L: Surgery-associated acquired hemophilia A. Ann Hematol. 80:691–693. 2011.
15	Bouvry P and Recloux P: Acquired hemophilia. Haematologica. 79:550–556. 1994.PubMed/NCBI
16	Sborov DW and Rodgers GM: Acquired hemophilia A: A current review of autoantibody disease. Clinical Advances in Hematol Oncol. 10:19–27. 2012.
17	Delgado J, Jimenez-Yuste V, Hernandez-Navarro F and Villar A: Acquired haemophilia: Review and meta-analysis focused on therapy and prognostic factors. Br J Haematol. 121:21–35. 2003. View Article : Google Scholar : PubMed/NCBI
18	Collins PW and Percy CL: Advances in the understanding of acquired haemophilia A: Implications for clinical practice. Br J Haematol. 148:183–194. 2010. View Article : Google Scholar : PubMed/NCBI
19	Shetty S, Bhave M and Ghosh K: Acquired hemophilia A: Diagnosis, aetiology, clinical spectrum and treatment options. Autoimmun Rev. 10:311–316. 2011. View Article : Google Scholar : PubMed/NCBI
20	Federici AB, Budde U, Castaman G, Rand JH and Tiede A: Current diagnostic and therapeutic approaches to patients with acquired von Willebrand syndrome: A 2013 update. Semin Thromb Hemost. 39:191–201. 2013. View Article : Google Scholar : PubMed/NCBI
21	Tanaka I, Amano K, Taki M, Oka T, Sakai M, Shirahata A, Takata N, Takamatsu J, Taketani H, Hanabusa H, et al: A 3-year consecutive survey on current status of acquired inhibitors against coagulation factors in Japan: Analysis of prognostic factors. Japanese J Thromb Hemost. 19:140–153. 2008. View Article : Google Scholar