A new case series and literature review suggests acquired hemophilia (AH) should be suspected in patients who present with rheumatic diseases (RDs), spontaneous bleeding, and prolonged activated partial thromboplastin time (APTT).
The study, published in the Journal of Inflammation Research, explored the association between AH and RDs, as well as the disease progression and treatment.
“To strengthen the understanding of [RDs] as the most common underlying conditions associated with [AH], a potentially fatal bleeding condition due to the development of autoantibodies or inhibitors to coagulation factor VIII, and rarely to factor IX, here we presented two cases of RDs associated [with] AH to elucidate the disease progression, treatment, and prognosis,” the authors explained.
The 2 cases presented involved a 60-year-old woman and a 67-year-old man, both presenting with ecchymosis of the limbs.
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The woman was diagnosed with systemic lupus erythematosus, secondary Sjogren syndrome, and AH. She was successfully treated with 400 mg/kg/day of intravenous (IV) immunoglobulin for 5 days, 2 mg/kg/day of IV methylprednisolone (mPSN), 200 mg of oral hydroxychloroquine (HCQ) twice a day, 50 mg of cyclosporin A (CsA) twice a day, and 600 mg in total of IV cyclophosphamide (CTX) for 15 days until her symptoms resolved.
She continued to receive oral mPSN, HCQ, and CsA after discharge. She had no recurrence at the 2-year follow-up.
The man was diagnosed with undifferentiated connective tissue disease associated with AH. He was treated with 1 mg/kg/day of IV mPSN and 600 mg of CTX as initial therapy, and he continued to receive oral mPSN gradually tapered from 40 mg/day to 8 mg/day and 200 mg of HCQ twice a day as maintenance therapy. His symptoms had considerably improved at the 1-year follow-up.
The presented cases along with data from the literature led the authors to recommend that platelet counts and clotting tests be performed as soon as possible in patients with RDs and bleeding symptoms. If AH is confirmed, interventions to stop the bleeding and reduce inhibitors are essential for successful treatment.
Tang Q, Liao J, Xie X. Acquired hemophilia associated with rheumatic diseases: a case-based systematic review. J Inflamm Res. 2022;15:4385-4393. doi:10.2147/JIR.S369288