The occurrence of the antibody against factor VIII is a well-known phenomenon in hemophilia A, occuring in 5 to 15% of the hemophilic patients in the United States, England, Sweden and France. The development of factor VIII in non-hemophilic patients is rare and may occur in healthy individuals, mostly elderly and women in postpartum period, and in patients with malignant neoplasia or autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis and Sjögren's syndrome. We described the case of a 64-year-old female patient who has had seropositive rheumatoid arthritis for 23 years and presented with a ten-day history of progressive ecchymosis. Therapy with methylprednisolone, intravenous cyclophosphamide, immunoglobulin and factor VIII reposition was instituted, resulting in a remission of the bleeding and negativity for antibodies against factor VIII titers. We concluded that, despite its rarity, the presence of acquired factor VIII inhibitors should be investigated when patients with autoimmune diseases develop bleeding manifestations.
The clinical condition deteriorated with recrudescence and exacerbation of the abdominal pain. Blood analysis showed a pronounced elevation of CRP 418mg/L, amylase 108U/L, lipase 242 U/L, maintaining the hyponatremia, with normal TSH (Table1). It is relevant to refer that the blood sample was lipemic, with the standing plasma containing a cream layer over a turbid layer. Blood cultures were collected and empiric antibiotic therapy with Piperacillin-Tazobactam started. Further study included abdominal computed tomography ( Fig. 2), relating signs of acute pancreatitis as well as small size gallstones. The patient received treatment with intravenous fluids, fasting and pain control, obtaining clinical improvement. Later we acknowledged the lipid profile results: total cholesterol 1282mg/ dl, HDL 40mg/dl, TG 6221mg/dl. Medical therapy with rosuvastatin 40mg and fenofibrate 267mg was started. During the hospital stay the patient evolved with no complications and no organic dysfunction and after three weeks she was discharged, with TG 994mg/dl. With the diagnosis of type V hyperlipoproteinemia in Fredrickson classification (based on the examination of standing plasma and Total cholesterol/TG ratio) 8,9 , she was referred to genetic testing, but no abnormalities were found. After discharge, the patient presented wavering TG in relation with inappropriate compliance, TG varying from 299 to 5825mg/dl. She had another admission with the diagnosis of pancreatitis and after 6 months she was submitted to cholecystectomy. Two months after surgery the patient had another recurrence of pancreatitis, with TG 5444mg/dl. A magnetic resonance was performed and the possibility of residual choledocholithiasis was discarded.
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