J Rheum Dis.  2017 Oct;24(5):309-312. 10.4078/jrd.2017.24.5.309.

Acquired Hemophilia A Combined with Systemic Lupus Erythematosus: A Case Report and Literature Review

• You J
• Kim H
• Park JS
• Chang MH
• Lee CH

• KMID: 2394347
• DOI: http://doi.org/10.4078/jrd.2017.24.5.309

Abstract

Acquired hemophilia A (AHA) is a rare hemorrhagic disorder caused by autoantibodies against factor VIII (FVIII). An 80-year-old woman presented multiple bruises on her upper and lower extremities, along with gross hematuria. Extensive ecchymosis and swelling were observed on the buttocks. She had anemia and normal platelet count. The initial coagulation results showed prolonged activated partial thromboplastin time (aPTT, 68.5 seconds) and normal prothrombin time. According to the mixing test, we observed a decreased FVIII activity (2%), increased factor VIII inhibitor (FVIII-I) titer (74.4 BU), and negative lupus anticoagulant. AHA was diagnosed based on late onset bleeding and increased FVIII-I titer. Additionally, she met the criteria for systemic lupus erythematosus (oral ulcer, photosensitivity, renal disorder, and positivity for antinuclear and anti-β2-glycoprotein-I antibodies). She was started on oral prednisolone for FVIII-I eradication. Post-treatment, her bleeding tendency, aPTT (47.3 seconds), and FVIII-I titer decreased (1.24 BU), and FVIII activity increased (10%).

Keyword

Hemophilia A; Acquired; Systemic lupus erythematosus

MeSH Terms

Aged, 80 and over
Anemia
Autoantibodies
Buttocks
Contusions
Ecchymosis
Factor VIII
Female
Hematuria
Hemophilia A*
Hemorrhage
Hemorrhagic Disorders
Humans
Lower Extremity
Lupus Coagulation Inhibitor
Lupus Erythematosus, Systemic*
Partial Thromboplastin Time
Platelet Count
Prednisolone
Prothrombin Time
Ulcer
Autoantibodies
Factor VIII
Lupus Coagulation Inhibitor
Prednisolone

Reference

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

2. Lafferty TE, Smith JB, Schuster SJ, DeHoratius RJ. Treatment of acquired factor VIII inhibitor using intravenous immunoglobulin in two patients with systemic lupus erythematosus. Arthritis Rheum. 1997; 40:775–8.

3. Trotta F, Bajocchi G, La Corte R, Moratelli S, Sun LY. Long-lasting remission and successful treatment of acquired factor VIII inhibitors using cyclophosphamide in a patient with systemic lupus erythematosus. Rheumatology (Oxford). 1999; 38:1007–9.

4. Ishikawa T, Tsukamoto N, Suto M, Uchiumi H, Mitsuhashi H, Yokohama A, et al. Acquired hemophilia A in a patient with systemic lupus erythematosus. Intern Med. 2001; 40:541–3.

5. Onishi S, Hojo N, Sakai I, Yasukawa M, Hato T, Minamoto Y, et al. Rupture of the gallbladder in a patient with acquired factor VIII inhibitors and systemic lupus erythematosus. Intern Med. 2004; 43:1073–7.

6. Porru G, Mura V, Piga M, Ibba V, Vacca A, Cauli A, et al. Hemarthrosis as acute presentation of acquired hemophilia in a patient with systemic lupus erythematosus: successful treatment and long-lasting remission. Clin Rheumatol. 2008; 27:1581–4.

7. Akahoshi M, Aizawa K, Nagano S, Inoue H, Sadanaga A, Arinobu Y, et al. Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review. Mod Rheumatol. 2008; 18:511–5.

8. Rezaieyazdi Z, Sharifi-Doloui D, Hashemzadeh K, Shirdel A, Mansouritorghabeh H. Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus; review of literature. Blood Coagul Fibrinolysis. 2012; 23:71–4.

9. Dicke C, Holstein K, Schneppenheim S, Dittmer R, Schneppenheim R, Bokemeyer C, et al. Acquired hemophilia A and von Willebrand syndrome in a patient with late-onset systemic lupus erythematosus. Exp Hematol Oncol. 2014; 3:21.

10. Collins PW. Therapeutic challenges in acquired factor VIII deficiency. Hematology Am Soc Hematol Educ Program. 2012; 2012; 369–74.

11. Franchini M, Lippi G. Acquired factor VIII inhibitors. Blood. 2008; 112:250–5.

12. Morrison AE, Ludlam CA. Acquired haemophilia and its management. Br J Haematol. 1995; 89:231–6.

13. 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; 121:21–35.

14. Jung KH, Choi JH, Lee HS. Acquired hemophilia in a patient with rheumatoid arthritis. J Korean Rheum Assoc. 2010; 17:295–300.