Hamostaseologie 2019; 39(01): 095-099
DOI: 10.1055/s-0038-1668570
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Acquired Haemophilia A Associated with Subsequent Hepatocellular Carcinoma

Renata Pacholczak
1   Department of Anatomy, Jagiellonian University Medical College, Cracow, Poland
2   Centre of Oncology, Maria Sklodowska-Curie Memorial Institute, Cracow Branch, Poland
,
Stanisława Bazan-Socha
3   Department of Internal Medicine, Jagiellonian University Medical College, Cracow, Poland
,
Joanna Zdziarska
4   Department of Hematology, Jagiellonian University Medical College, Cracow, Poland
,
Teresa Iwaniec
3   Department of Internal Medicine, Jagiellonian University Medical College, Cracow, Poland
,
Jerzy A. Walocha
1   Department of Anatomy, Jagiellonian University Medical College, Cracow, Poland
,
Jacek Musiał
3   Department of Internal Medicine, Jagiellonian University Medical College, Cracow, Poland
,
Jerzy Dropiński
3   Department of Internal Medicine, Jagiellonian University Medical College, Cracow, Poland
› Author Affiliations
Further Information

Publication History

20 January 2018

23 May 2018

Publication Date:
15 August 2018 (online)

Abstract

Acquired haemophilia A (AHA) is a rare autoimmune disease caused by antibodies directed against clotting factor VIII. About half of cases are idiopathic, but AHA may also be secondary to autoimmune, dermatologic, or oncologic diseases. In approximately 10% of non-idiopathic cases, the disease occurs after or with the diagnosis of cancer as an extremely rare paraneoplastic syndrome. We describe the case of a 73-year-old male patient diagnosed with AHA and successfully treated with recombinant human activated factor VIIa and immunosuppression. Two and a half years later, however, the disease relapsed and a routine ultrasound revealed a liver tumour that was then diagnosed as hepatocellular carcinoma. We present this case to increase awareness that this life-threatening condition may develop years prior to the diagnosis of cancer.

Authors' Contribution

Renata Pacholczak: conception of study, study design, acquisition of data, data analysis and interpretation, drafting of manuscript.


Stanisława Bazan-Socha: data analysis and interpretation, revising of manuscript.


Joanna Zdziarska: acquisition of data, revising of manuscript.


Teresa Iwaniec: data analysis and interpretation, revising of manuscript.


Jerzy A. Walocha: revising of manuscript, general supervision.


Jacek Musiał: revising of manuscript, general supervision.


Jerzy Dropiński: revising of manuscript, general supervision.


 
  • References

  • 1 Franchini M, Gandini G, Di Paolantonio T, Mariani G. Acquired hemophilia A: a concise review. Am J Hematol 2005; 80 (01) 55-63
  • 2 Zdziarska J, Musiał J. Acquired hemophilia A: an underdiagnosed, severe bleeding disorder. Pol Arch Med Wewn 2014; 124 (04) 200-206
  • 3 Collins PW, Hirsch S, Baglin TP. , et al; 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 (05) 1870-1877
  • 4 Knoebl P, Marco P, Baudo F. , et al; EACH2 Registry Contributors. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost 2012; 10 (04) 622-631
  • 5 Tengborn L, Baudo F, Huth-Kühne A. , et al; EACH2 Registry Contributors. Pregnancy-associated acquired haemophilia A: results from the European Acquired Haemophilia (EACH2) registry. BJOG 2012; 119 (12) 1529-1537
  • 6 Sakurai Y, Takeda T. Acquired hemophilia A: a frequently overlooked autoimmune hemorrhagic disorder. J Immunol Res 2014; 2014: 320674
  • 7 Shetty S, Bhave M, Ghosh K. Acquired hemophilia a: diagnosis, aetiology, clinical spectrum and treatment options. Autoimmun Rev 2011; 10 (06) 311-316
  • 8 Bazan-Socha S, Zdziarska J, Kleczyńska W, Iwaniec T, Musiał J. Nabyta hemofilia A u pacjentki z reumatoidalnym zapaleniem stawów. Reumatologia 2011; 49 (06) 446-449
  • 9 Huang SY, Tsay W, Lin SY, Hsu SC, Hung MH, Shen MC. A study of 65 patients with acquired hemophilia A in Taiwan. J Formos Med Assoc 2015; 114 (04) 321-327
  • 10 Franchini M, Mannucci PM. Acquired haemophilia A: a 2013 update. Thromb Haemost 2013; 110 (06) 1114-1120
  • 11 Reeves BN, Key NS. Acquired hemophilia in malignancy. Thromb Res 2012; 129 (Suppl. 01) S66-S68
  • 12 Shetty SD, Ghosh K. Challenges and open issues in the management of acquired hemophilia A (AHA). Blood Cells Mol Dis 2015; 54 (03) 275-280
  • 13 Girardi DdaM, Silva DR, Villaça PR. , et al. Acquired hemophilia A in a patient with advanced prostate cancer. Autops Case Rep 2015; 5 (02) 55-59
  • 14 Ichikawa S, Kohata K, Okitsu Y. , et al. Acquired hemophilia A with sigmoid colon cancer: successful treatment with rituximab followed by sigmoidectomy. Int J Hematol 2009; 90 (01) 33-36
  • 15 Sheth C, Gill A, Sekhon S. Life-threatening hemorrhage from acquired hemophilia A as a presenting manifestation of prostate cancer. J Community Hosp Intern Med Perspect 2016; 6 (04) 32461
  • 16 Hauser I, Lechner K. Solid tumors and factor VIII antibodies. Thromb Haemost 1999; 82 (03) 1005-1007
  • 17 van Durme CM, Idema RN, van Guldener C. Two rare complications of glioblastoma multiforme: persistent hiccup and acquired haemophilia A. Neth J Med 2008; 66 (07) 286-288
  • 18 Franco-Moreno AI, Santero-García M, Cabezón-Gutiérrez L, Martín-Díaz RM, García-Navarro MJ. Acquired hemophilia A in a patient with hepatocellular carcinoma: a case report and literature review. Ann Hematol 2016; 95 (12) 2099-2100
  • 19 Croft J, Sahu S. Acquired haemophilia A in hepatocellular carcinoma. Br J Haematol 2014; 164 (05) 617-617
  • 20 Neilson RF, Walker ID, Robertson M. Factor VIII inhibitor associated with hepatocellular carcinoma. Clin Lab Haematol 1993; 15 (02) 145-148
  • 21 Scandella D, Kessler C, Esmon P. , et al. Epitope specificity and functional characterization of factor VIII inhibitors. Adv Exp Med Biol 1995; 386: 47-63
  • 22 Sallah S, Singh P, Hanrahan LR. Antibodies against factor VIII in patients with solid tumors: successful treatment of cancer may suppress inhibitor formation. Haemostasis 1998; 28 (05) 244-249
  • 23 Escobar MA. Bleeding in the patient with a malignancy: is it an acquired factor VIII inhibitor?. Cancer 2012; 118 (02) 312-320
  • 24 Sallah S, Nguyen NP, Abdallah JM, Hanrahan LR. Acquired hemophilia in patients with hematologic malignancies. Arch Pathol Lab Med 2000; 124 (05) 730-734
  • 25 Sallah S, Wan JY. Inhibitors against factor VIII in patients with cancer. Analysis of 41 patients. Cancer 2001; 91 (06) 1067-1074
  • 26 Biron-Andreani C, de Moerloose P, D'oiron R, Chambost H, Schved JF, Hermans C. Cancer detection and management in patients with haemophilia: a retrospective European multicentre study. Haemophilia 2014; 20 (01) 78-82
  • 27 Belt RJ, Leite C, Haas CD, Stephens RL. Incidence of hemorrhagic complications in patients with cancer. JAMA 1978; 239 (24) 2571-2574
  • 28 Huth-Kühne A, Baudo F, Collins P. , et al. International recommendations on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica 2009; 94 (04) 566-575
  • 29 Tiede A, Klamroth R, Scharf RE. , et al. Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study. Blood 2015; 125 (07) 1091-1097
  • 30 Sborov DW, Rodgers GM. How I manage patients with acquired haemophilia A. Br J Haematol 2013; 161 (02) 157-165
  • 31 Collins P, Baudo F, Huth-Kühne A. , et al. Consensus recommendations for the diagnosis and treatment of acquired hemophilia A. BMC Res Notes 2010; 3: 161