Pseudo tumours in haemophilia patients

Pseudo tumours are amongst the rare yet pathognomonic complications of haemophilia. They are old, encapsulated haematomas which due to their sometimes enormous size can cause massive complaints. These haematomas are surrounded by a thick fibrous capsule. They are attributed to persistent bleedings. The pathophysiology of pseudo tumors is not conclusively established yet. Some believe that they originate from bone material or the periosteum, while others suggest their development from soft tissue. They spread aggressively, displace the surrounding tissue, and cause secondary periosteal erosion of the bone. This results in bone resorption and destruction of surrounding muscular and soft tissue. Pseudo tumours develop slowly over many years. They occur primarily in adults and are largely unresponsive to conservative treatment. Case: A 48-year-old man with moderate hemophilia A (FVIII: C 2%) and no FVIII inhibitor. Due to recurrent bleeding into the muscle of the right thigh diagnosis of two pseudo tumours (psoas, adductor magnus). In 2004 tumour extirpation with subsequent relapse; because of high local bleeding tendency (despite permanent prophylaxis with FVIII concentrate and adjusted lifestyle) surgical revision in 02/2008. Postoperatively, no recurrent bleeding; the patient is fully fit for work three months later. Conclusion: in order to reduce the complication rate when a pseudo tumor is suspected, patients should be treated in a specially equipped interdisciplinary center with adequately trained and experienced surgeons and haemostaseologists.