Iliopsoas pseudotumour is a serious complication of haemophilia. We present the case of a 20-year-old male patient with a six-month history of left leg weakness, limitation of movement and wasting of the muscles. Clinically he was diagnosed as having a psoas muscle rhabdomyosarcoma. During a computed tomography (CT) scan-guided Tru-cut biopsy he developed a serious and life-threatening bleeding...

A trial of prophylactic replacement therapy in low dosage once a week is described in two patients with classical haemophilia and one patient with Christmas disease, using concentrates of factor VIII and factor IX respectively. The clinical effectiveness and complications of the therapy are assessed and discussed. It was concluded that the patient suffering from Christmas disease showed both ob...

The seventh edition of the haemophilia B database lists in easily accessible form all known factor IX mutations due to small changes (base substitutions and short additions and/or deletions of <30 bp) identified in haemophilia B patients. The 1535 patient entries are ordered by the nucleotide number of their mutation. Where known, details are given on: factor IX activity, factor IX antigen in c...

The aim of this paper is to present and discuss psychological care for children with haemophilia. The article identifies and indicates specific features of the psychosocial functioning of these patients, which need to be further investigated to gain a better understanding of the difficulties encountered by haemophiliacs and their families. Understanding these difficulties in the context of a ch...

Unresponsiveness to skin testing with PPD and tetanus toxoid was commonly seen in patients with haemophilia A but not infected with human immunodeficiency virus but was uncommon in controls. Vaccination history indicated that the unresponsive patients had not been immunised in childhood. Other tests of immune competence (skin tests with other antigens, lymphocyte stimulation with mitogens and a...

an account of the management of a haemophilic patient is presented who developed severe, prolonged hacmoptyscs caused by hydatid lung disease, and who ultima tely requ ired thoracotomy and lobec tomy of the lungs.

Medication non-adherence is a true public health problem. Despite advancements in the molecular understanding of disease and improvements in therapy, patient health outcomes will not improve unless patients take prescribed medications. Decreasing the gap between efficacious and effective therapy for patients with haemophilia is an essential research agenda. Adherence is particularly important f...

Haemophilia A is a rare genetic condition leading to coagulation factor VIII deficiency and thus predisposing to bleeding diathesis. Due to advances in treatment, life expectancy of haemophilia A sufferers is increasing, and the incidence and prevalence of coronary artery disease are rising. There have been many reported cases of acute myocardial infarction in such patients, who subsequently un...

This review focuses on the use of recombinant porcine factor VIII (FVIII) for the treatment of bleeding episodes in patients with severe haemophilia A complicated by inhibitors and in those with acquired haemophilia A due to the onset of anti-FVIII autoantibodies. We present the main characteristics of recombinant porcine factor VIII (FVIII) and provide a summary of the published results of the...

Many rapid advances have been made in the diagnosis and therapy of haemophilia. Nevertheless, the condition still poses problems and challenges (e.g., joint disease, transfusion-transmitted diseases, inhibitors, provision of care in developing countries, and education and cost issues). WHO and the World Federation of Hemophilia held a joint meeting in Geneva, on 21-23 March 1994, to discuss and...