Acquired haemophilia A in a woman with autoimmune hepatitis and systemic lupus erythematosus

Abstract

Acquired haemophilia A, secondary to systemic lupus erythematosus (SLE), is a rare bleeding diathesis. Here we report a 37-year-old woman with autoimmune hepatitis who developed SLE and acquired haemophilia caused by factor VIII (fVIII) inhibitors. She presented with spontaneous ecchymosis and haematuria. There were a prolongation of the activated partial thromboplastin time, reduced fVIII activity and a high titre of fVIII inhibitors. Therapeutic regimen was started with intravenous methylprednisolone pulse, continued with prednisolone, intravenous pulse cyclophosphamide and fresh frozen plasma. After 8 weeks, fVIII inhibitor assay was negative.

Acquired Hemophilia Associated with Rheumatic Diseases: A Case-Based Systematic Review

“… Arthanari et al 4 78y/F Caucasion Spontaneous subcutaneous bleeding APTT 56s; FVIII inhibitor 22 BU; FVIII activity 3%; RF+, ANA+ RA CTX 100mg daily, Transfusion of aPCC Died less than 2 months after diagnosis due to mass bleeding and multiorgan failure. Rezaieyazdi et al 5 37y/F UK Fatigue, malaise, abdominal pain, haematuria, large ecchymotic area on the left flank and leukopenia HBG 12.5g/dl; PLT 180×10 3 /µL; WBC 3×10 3 /µL; APTT 95s; FVIII activity 3.9%; FVIII inhibitors>200 BU; ANA+, anti-Sm+, anti small nuclear RNP+, anti-dsDNA+; proteinuria 2350mg per 24h SLE Fresh frozen plasma infusion (10mL/kg), IV mPSN pulse 1000mg/day for 3 days, continued with 60mg PSN per day, IV pulse CTX APTT, FVIII activity, and FVIII inhibitors became normal after 4 weeks. No relapse during the 1.5-year follow-up.…”

“…After excluding reviews, duplicates, and articles with too little clinical data, a total of 14 articles were included in the final literature review. [3][4][5][6][7][8][9][10][11][12][13][14][15][16] Detailed characteristics were listed in Table 1. SLE (7/14) and rheumatoid arthritis (RA) (4/14) were the most common concurrent RDs.…”