Summary.  von Willebrand disease (VWD) is a bleeding disorder caused by inherited defects in the concentration, structure, or function of von Willebrand factor (VWF). VWD is classified into three primary categories. Type 1 includes partial quantitative deficiency, type 2 includes qualitative defects, and type 3 includes virtually complete deficiency of VWF. VWD type 2 is divided into four secondary categories. Type 2A includes variants with decreased platelet adhesion caused by selective deficiency of high‐molecular‐weight VWF multimers. Type 2B includes variants with increased affinity for platelet glycoprotein Ib. Type 2M includes variants with markedly defective platelet adhesion despite a relatively normal size distribution of VWF multimers. Type 2N includes variants with markedly decreased affinity for factor VIII. These six categories of VWD correlate with important clinical features and therapeutic requirements. Some VWF gene mutations, alone or in combination, have complex effects and give rise to mixed VWD phenotypes. Certain VWD types, especially type 1 and type 2A, encompass several pathophysiologic mechanisms that sometimes can be distinguished by appropriate laboratory studies. The clinical significance of this heterogeneity is under investigation, which may support further subdivision of VWD type 1 or type 2A in the future.

SummaryA multicentre retrospective survey was conducted to re-assess the use of porcine factor VIII (HYATE:C), its side effects and the selection of patients for regular or home-therapy. 15,152,000 units of HYATE:C were used by 154 patients. The median inhibitor cross-reactivity to porcine VIIIC of 137 patients was 15%, 27% of patients lacking cross-reactivity. An absent, intermediate or brisk specific antiporcine anamnestic response was observed in 29, 40 and 31% of patients respectively. Seven patients were treated on-demand as home-therapy for a median 6.2, range 1.5-13 years. 23 further patients were treated regularly in hospital for a median of 3, range 2-7 years. This group used 8,319,000 U of porcine VIIIC for 2,000 bleeding episodes.The incidence of transfusion reactions was 0.001%, 0.64% and 2.3%, for domiciliary infusions, infusions in multiply treated inpatients, and unselected in-patient infusions, respectively. The risk of reactions was dose-related. A post-infusion fall in platelet count was common, but usually transient and clinically insignificant. This was also dose-related (r = -0.64, p = 0.002). Marked reductions in platelet count were occasionally seen, usually with intensive replacement therapy. The relative lack of side effects observed amongst patients treated at home is attributable to the low, median 33 U/kg, dose used by this group.A subgroup of inhibitor patients, identifiable by their absent or modest anamnestic response to porcine factor VIII may be treated regularly and safely with this product in small doses, over a period of years.

The overall purpose of the 'Decade Plan' published in 1992 was to do everything possible to make effective treatment available to people with haemophilia throughout the world. In 1990 it was estimated that there were 350,000 individuals with haemophilia worldwide, with 80% or 280,000 without treatment. By the year 2020 this may have reached the figure 440,000, or the population of Jerusalem. Earlier this century Carroll Birch reported a series of 113 patients where 82 died before their 15th year, and only six survived beyond 40. Thus as Jones has pointed out for the developing world this is a paediatric problem for the WFH to face now. The examples described in this paper illustrating the WFH programmes and the commitment of many people from the 88 member organisations worldwide as well as the doctors and scientists shows what can be done through cooperation. There is no doubt that these achievements are capitalising from the unique quality of WFH from the beginning, that is a membership which includes both those with haemophilia and their families, and those with an interest in treating haemophilia.

We report two cases of this rare postoperative condition. Both patients having undergone extensive surgery to their hips, developed increased bone growth in the soft tissue of the hip, accounting for the reduced range of movement, pain and stiffness. Plain x-rays taken confirmed the presence of heterotopic ossification, also known as a variant of myositis ossificans. We review the literature and discuss treatment options of this rare condition in patients with end-stage haemophilic arthropathy.

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