[Haemophilic pseudotumour of the distal femur - a case report and characterisation of this entity]

Haemophilic pseudotumour is a rare disease occurring in 1 % of patients with severe haemophilia as a long-term complication. Because both haemophilia A (factor VIII deficiency) and haemophilia B (factor IX deficiency) are X-linked recessive genetic disorders, pseudotumours are found almost exclusively in men between 20 and 70 years of age. Haemophilic pseudotumour has been defined as a progressive cystic swelling, produced by recurrent haemorrhage, which exerts increasing pressure on nearby structures. As a result, necrosis of skin, muscle and bone can be found. Neurovascular obstruction as well as pathologic fractures are severe complications of this disease. Most patients with pseudotumours are asymptomatic for a long time and many patients report sustaining an injury prior to the development of the tumour. Most haemophilic pseudotumours of the bone are located in the pelvis, femur and hand. We present the case of a 59-year-old male patient suffering from a haemophilic pseudotumour of the right distal femur. After verification of the diagnosis by means of an open biopsy, final surgery with curettage and plombage with bone cement was performed.     «

Haemophilic pseudotumour is a rare disease occurring in 1 % of patients with severe haemophilia as a long-term complication. Because both haemophilia A (factor VIII deficiency) and haemophilia B (factor IX deficiency) are X-linked recessive genetic disorders, pseudotumours are found almost exclusively in men between 20 and 70 years of age. Haemophilic pseudotumour has been defined as a progressive cystic swelling, produced by recurrent haemorrhage, which exerts increasing pressure on nearby stru...     »