Acquired haemophilia A in an elderly patient related to relapsed cervix carcinoma

Open access

Abstract

Acquired haemophilia A (AHA) is a rare, autoimmune disease, presenting as sudden haemorrhages without any personal or family history. Anti-factor VIII (FVIII) is the most commonly recognised autoantibody resulting in decreased factor activity. The aetiology and pathophysiology of these antibodies remains unclear. Approximately 50% of reported cases are idiopathic; the rest are associated with other conditions, mainly underlying malignancies, autoimmune diseases (eg rheumatoid arthritis (RA), systemic lupus erythematosus), drug administration and postpartum period. A 74-year-old woman presented to our institution with haematochezia and haematuria. She had a medical history of cervix carcinoma; total abdominal hysterectomy and bilateral salpingo-oophorectomy with postoperative chemoradiotherapy was performed in 2011. She had also been followed up for 20 years for deforming and severe RA, which was in low-disease activity with methotrexate and corticosteroid. Laboratory investigations for abnormal bleeding revealed prolongation of activated partial thromboplastin time (APTT). APTT prolongation was not corrected by 50:50 plasma mixing studies, and a confirmatory factor assay demonstrated FVIII deficiency (1.4 IU/dL; normal range 50-150 IU/dL). Positive FVIII antibodies on Bethesda testing confirmed a diagnosis of AHA. A rectosigmoid mass and fistula between rectum and bladder were discovered by computed tomography (CT). Bleeding was controlled with recombinant activated factor VII (rFVIIa) after two weeks. Eradication of the inhibitor was achieved with high-dose pulse methylprednisolone for two days and then 2mg/kg daily over four weeks.

1. Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost 1981; 45(3): 200-3.

2. Cohen AJ, Kessler CM. Acquired inhibitors. Baillieres Clin Haematol 1996; 9(2): 331-54.

3. Knoebl P, Marco P, Baudo F, et al. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost 2012; 10(4): 622-31.

4. 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.

5. Bouvry P, Recloux P. Acquired hemophilia. Haematologica 1994; 79(6): 550-6.

6. Yee TT, Taher A, Pasi KJ, Lee CA. A survey of patients with acquired haemophilia in a haemophilia centre over a 28-year period. Clin Lab Haematol 2000; 22(5): 275-8.

7. Franchini M, Gandini G, Di Paolantonio T, Mariani G. Acquired hemophilia A: a concise review. Am J Hematol 2005; 80(1): 55-63.

8. Hay CR. Acquired haemophilia. Baillieres Clin Haematol 1998; 11(2): 287-303.

9. Sallah S, Wan JY. Inhibitors against factor VIII in patients with cancer. Analysis of 41 patients. Cancer 2001; 91(6): 1067-74.

10. O'Connor CR. Systematic review of the presentation of coagulation factor VIII inhibitors in rheumatic diseases: A potential cause of life-threatening hemorrhage. Semin Arthritis Rheum 2015; 44(6): 695-709.

11. Hay CR, Negrier C, Ludlam CA. The treatment of bleeding in acquired haemophilia with recombinant factor VIIa: a multicentre study. Thromb Haemost 1997; 78(6): 1463-7.

12. Lusher JM. Use of prothrombin complex concentrates in management of bleeding in hemophiliacs with inhibitors-- benefits and limitations. Semin Hematol 1994; 31(2 Suppl 4): 49-52.

13. Giangrande P. Acquired hemophilia. Revised edition. Treatment of hemophilia, no. 38. World Federation of Hemophilia, 2012. Available from http://www1.wfh.org/publication/files/pdf-1186.pdf (accessed 25 November 2018).

Journal Information

Metrics

All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 40 40 28
PDF Downloads 20 20 16