Summary. Two cases of von Willebrand's disease and angiodysplasia with intractable gastrointestinal bleeding are presented. Replacement therapy with cryoprecipitate and variable purity von Willebrand factor (VWF) was ineffective, as were other treatments including steroids, immunoglobulin and hormonal replacement. Both patients required massive blood transfusion and product support. The ef®cacy of somatostatin and an analogue is described. In one patient, we observed a rise in von Willebrand factor activity after octreotide infusion.
We report the case of a 26-year-old man with haemophilia B and human immunodeficiency virus infection (HIV) who presented with a short history of multiple dermatofibromas. He also had troublesome psoriasis for which he was receiving acitretin with UVB therapy and had recently discontinued low-dose daily prednisolone for associated arthropathy. Multiple dermatofibromas are rare and have been reported previously in association with autoimmune disease, especially in individuals receiving immunosuppressive therapy. We believe this to be the first report of multiple dermatofibromas in an individual with advanced HIV infection and propose that it may be related to his viral-mediated immunodeficiency, possibly augmented by UVB and systemic corticosteroid therapy.
Recent reports have suggested an association between Perthes' disease and an underlying thrombophilic or hypofibrinolytic tendency. In Northern Ireland there is a high incidence of Perthes' disease (11.7 per 100 000 or 1 in 607 children) in a stable paediatric population.We reviewed 139 children with Perthes' disease and compared them with a control group of 220 aged-and gender-matched healthy primary schoolchildren with similar racial and ethnic backgrounds.There were no significant deficiencies of antithrombotic factors protein C, protein S, antithrombin III or resistance to activated protein C. A total of 53 (38.1%) of the children with Perthes' disease had a prolonged activated partial thromboplastin time (> 38) compared with 13 (5.9%) of the control group (p < 0.001). Our findings have shown that using standard assays, thrombophilia secondary to antithrombotic factor deficiency or resistance to activated protein does not appear to be an aetiological factor for Perthes' disease. The cause of the prolonged activated partial thromboplastin time, usually associated with a clotting factor deficiency, is under further investigation.
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