Noninvasive Management of a Haemophilic Pseudotumor in a 17-year Old Male with Mild Heamophilia B

Haemophilic pseudotumor is a rare but feared complication in patients with severe haemophilia. A pseudotumor is an encapsulated blood formation within large muscles or nearby large joints. Over the past decades several treatment options have been described. Next to surgery, there are different kinds of non-invasive treatment strategies including factor substitution and antifibrinolytic therapy. However, there are rare reports of the use of tranexamic acid in patients with haemophilic pseudotumor. We’re reporting of a 17-year old male with known mild haemophilia B who suffered significant bleeding in the left iliopsoas muscle after minor trauma. He initially presented to our clinic with pain and paraesthesia in the left hip. MRT imaging showed a haematoma in the left iliopsoas muscle with an extension of 18 x 5 × 5 cm. We started a non-invasive therapy with intermittent substitution of factor IX supplemented by daily administration of tranexamic acid. Two months later the patient once again showed up at our clinic after a minor trauma to the left hip. This time a computed tomography was performed, which revealed a progression of the haematoma in the left iliopsoas muscle. The haematoma now showed an extension of 20 × 8,5 × 7,8 cm in size. We once again administered factor IX supplemented with tranexamic acid. To demonstrate the success of our treatment we regularly obtained ultrasound imaging. Six weeks after we boosted therapy the formation was significantly smaller with an extension of 10 × 5 × 5 cm. Furthermore after three months the size decreased to 8 × 3 × 4 cm. To our knowledge we are first to show that the combination of factor IX substitution and tranexamic acid is a valid therapy option in patients with haemophilic pseudotumor and mild haemophilia B.

No conflict of interest has been declared by the author(s).