Successful treatment of acquired hemophilia A associated with immune thrombocytopenia and joint hemarthrosis

Abstract

Introduction:Acquired hemophilia A (AHA) is a rare bleeding disease caused by autoantibodies against factor VIII (FVIII). Spontaneous bleeding symptoms usually affect the skin, musco, muscle, and internal organs, while joint hemarthrosis in AHA is an extremely rare manifestation. AHA may have an autoimmune cause and is often associated with autoimmune disease, but no demonstrable platelet impairment was found in AHA patients. We report a patient with AHA complicated with a right shoulder joint hemarthrosis and immune thrombocytopenia. The patient was treated with fresh frozen plasma (FFP) and human prothrombin complex concentrate (hPCC) to control the active bleeding. Simultaneously this patient firstly accepted cyclophosphamide combined with prednisone to eradicate the inhibitor, while the treatment effect of cyclophosphamide combined with prednisone was not satisfactory. At last, she was successfully treated through the use of an anti-CD20 monoclonal antibody.Conclusion:AHA is an autoimmune disease and can co-exist with immune cytopenia besides connective tissue disease (CTD). Joint hemarthrosis is not specific to congenital hemophilia and mainly related to the extent of prolonged aPTT and weight loading of joint in AHA. When the first-line therapy of cyclophosphamide combined with prednisone is not enough to eradicate the inhibitor, especially for a higher inhibitor titer, anti-CD20 monoclonal antibody could play an important role.

The diagnosis and Management of HIV Associated Acquired Hemophilia A: A Case Series From Durban, South Africa

“…Antifibrinolytic agents, for example, tranexamic acid may be used as adjunctive therapy however caution is advised in patients receiving bypassing agents (APCC), as there is an increased risk of thrombosis. 3 , 15 , 16 In our setting, all patients diagnosed with AHA are transferred to the Hemophilia care unit in KEH for definitive management of the acute bleed as bypassing agents are not easily available in the district hospitals.…”

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

“… Ghozlani et al 13 66y/M UK Spontaneous ecchymotic patches and hemarthrosis APTT 49s; FVIII inhibitors 19 BU/mL; RF and CCP positive RA IV mPSN 240 mg for 3 days followed up with MTX 20mg/week and PED 7.5 mg/day; Rit 1g/2 weeks for 2 times; rFVIIa infusion After 6 months, FVIII was 75% and the RA was in remission. Wei et al 14 40y/F Asia Oral cavity and nose mucosal bleeding, joint hemarthrosis HGB 49g/l, PLT 31*10^9/l; APTT 107.4s; FVIII inhibitor positive; Platelet associated antibody IgG positive ITP IVIG 2g/kg; PED 1mg/kg/day; IV CTX 2 mg/kg/day. After failure, Rit at 375mg/m2/week was added After 4 weeks of Rit, APTT, FVIII activity, and FVIII inhibitors became normal.…”

“…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.…”