A 55 year old women presented to the emergency department with a swollen, painful and warm calf. She reported minor trauma to her leg tree weeks earlier with formation of a hematoma, worsening since 10 days. She also complained of having frequent ecchymoses. She was discharged with the suspicion of a torn soleus muscle and analgesics were prescribed. Three months later, she presented with purpura and extensive ecchymoses, covering more than 40% of her body. Initial laboratory investigations showed normal platelet count, normal PT and prolonged APTT. As there was no previous personal or known family history of bleeding diathesis, an acquired coagulopathy was suspected. Coagulation factors were determined and revealed FVIII:C-activity of 1%. The FVIII inhibitor titer was 47 Bethesda units/ml, measured by the Nijmegen method, confirming the diagnosis of acquired haemophilia A (AHA). Autoimmune diseases and malignancy were excluded as were drugs known to be associated with AHA. In order to control acute bleeding, infusion of activated prothrombin complex concentrate, FVIII inhibitor bypassing agent (FEIBA), as started twice daily. Immuno-suppressive therapy, aiming to reduce the auto-antibodies, consisted of 64mg of methylprednisolone daily, reduced by 8mg per week, associated with off-label use of rituximab one weekly during 4 weeks. The inhibitor titer gradually decreased and APTT normalized. One month later she complained of a swollen face, amyotrophy and troubled alertness.Steroids were reduced to a lower weekly dose. 2.5 months after diagnosis FVIII:C-activity was 138% and all clinical manifestations had disappeared.
|Conference||7th International Symposium on Women's Health Issues in Thrombosis and Haemostasis|
|Period||3/03/17 → 5/03/17|