J Korean Rheum Assoc.  2010 Sep;17(3):295-300.

Acquired Hemophilia in a Patient with Rheumatoid Arthritis

• Jung KH
• Choi JH
• Lee HS

Abstract

Acquired hemophilia is a rare disease caused by an autoimmune reaction to coagulation factor VIII, The mortality rate of this disease is very high (8~22%). Clinical manifestations are different from congenital hemophilia. Various diseases are associated with acquired hemophilia, including autoimmune diseases such as systemic lupus erythematosus or rheumatoid arthritis (RA), tumors, inflammatory bowel disease, psoriasis, asthma, diabetes, acute hepatitis B or C, and drug reactions. However, the underlying cause is unknown in approximately 50% of cases. A few cases of acquired hemophilia with RA have been published. However, no cases have been reported in Korea. We had a patient with longstanding RA and acquired hemophilia who was suffering from upper and lower extremity purpura with a deep intramuscular hematoma. The patient was successfully treated using cyclophosphamide combined with steroid.

Keyword

Rheumatoid arthritis; Acquired hemophilia

MeSH Terms

Arthritis, Rheumatoid
Asthma
Autoimmune Diseases
Cyclophosphamide
Factor VIII
Hematoma
Hemophilia A
Hepatitis B
Humans
Inflammatory Bowel Diseases
Korea
Lower Extremity
Lupus Erythematosus, Systemic
Psoriasis
Purpura
Rare Diseases
Stress, Psychological
Cyclophosphamide
Factor VIII

Figure

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  Fig. 1. (A) Multiple petechiae and ecchymosis on both lower extremities and marked swelling of the left leg compared to the right leg. (B) Endoscopy showing deep gastric ulcer at the lesser curvature.

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  Fig. 2. (A) X-ray of the hand showing joint space narrowing and multifocal erosive changes at the bilateral radiocarpal, intercarpal, and carpometacarpal joints. Bony erosion at the left trapezium and right radial head (arrows). (B) X-ray of the foot showing multifocal bony erosions at the right fifth metatarsal head (arrow).

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  Fig. 3. Whole-body bone scan demonstrating increased uptake into multiple joints of both hands, both wrists, left elbow, right ankle, the medial aspect of the left knee joint, and both hip joints.

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  Fig. 4. Ultrasonographic findings showing intramuscular hematoma between the soleus muscle and the gastrocnemius muscle of the left leg (arrow indicates intramuscular hematoma).

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  Fig. 5. Changes in activated partial thromboplastin time (aPTT), factor VIII, and factor VIII inhibitor during treatment.

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