SummaryThe incidence of factor VIII inhibitor was studied in a cohort of 56 previously untreated patients with severe hemophilia A (factor VIII below 1 U/dl). They received only one brand of highly purified factor VIII concentrate (HPSD-VIII) prepared by conventional chromatography with a solvent-detergent step for viral inactivation. Followup since the first infusion of HPSD-VIII was from 1 to 76 months (mean = 29) and cumulative exposure days (CED) from 1 to over 100 (median = 26). Five patients (9%) developed an inhibitor after 6 to 19 CED, only one being a high responder (2%), showing a low incidence of inhibitor compared with previous studies using high purity plasma- derived or recombinant products.

This report concerns 34 cases of heparin-associated thrombocytopenia in which standard heparin has been systematically replaced by LMW heparin CY 216 Choay. There were 21 women and 13 men, mean age, 69 years. Twenty-six of the 34 cases had thrombotic complications. All of the patients were treated by standard heparin of porcine mucosal origin, in most cases for prevention of deep vein thrombosis and pulmonary embolism. Twenty-six cases occurred along with orthopedic and traumatologic surgery (especially for total hip replacement, 15 cases). Four cases involved vascular surgery and three involved medical thrombotic disease. Standard heparin treatment was always replaced by LMW heparin CY 216. Initial doses were 0.30 ml three times daily, later increased to obtain global hypocoagulability. Surgical procedures (embolectomy and vena cava filter) were carried out when necessary. Five patients had thrombolytic treatment (urokinase) in conjunction with LMW heparin. The overall results were 31 recoveries, but with seven severe sequelae (three amputations and four hemiplegias) most often attributable to the first ischemic attack, and three deaths. For the last 16 patients, indirect platelet aggregation tests were performed (control platelet-rich plasma plus patient platelet-poor plasma plus LMW heparin): nine cases had negative tests, with nine recoveries and seven cases had positive tests, with five recoveries and two deaths.(ABSTRACT TRUNCATED AT 250 WORDS)

Several studies have reported the spontaneous loss of hepatitis C virus (HCV) antibodies in HCVexposed persons. However, the relationship between seroreversion and spontaneous virus clearance has yet to be precisely determined in a single homogeneous population of untreated immunocompetent patients. In this study, 32 human immunodeficiency virus -seronegative hemophiliacs who had been exposed to HCV were followed for a mean duration of 141 months; 22 remained chronic carriers (68.8%). All but 1 of the nonviremic patients (90.0%) showed partial (8 cases) or complete (2 cases) seroreversion. In contrast, all but 1 of the viremic patients (95.1%) had a stable serologic profile when analyzed by a recombinant immunoblot assay. The results indicate that any HCV antibody -positive immunocompetent patient with no detectable serum HCV RNA and normal alanine aminotransferase values and whose serial samples show a progressive decrease in the level of HCV antibodies present may be considered as having a resolved infection. Hepatitis C virus (HCV) infection is often characterized byPatients and Methods a chronic carriage of the virus, leading to chronic hepatitis, cirrhosis, and liver cell carcinoma [1,2]. It is frequently asPatients. One hundred seven hemophiliacs were followed as sumed that at least 50% of exposed persons remain chronically outpatients between July 1995 and December 1996 in our hemophilia treatment center. As the aim of this study was to analyze infected after primary infection [1]. Initial diagnosis of HCV serologic changes in HCV antibody levels with disease progresinfection is by detection of HCV antibody by EIA. This techsion, we retained only patients who had been followed for at least nique has been greatly improved over the last few years [3,4]. pearance of HCV antibodies may be predictive of self-limited For every patient, whether positive or negative for HCV antiinfection [6 -9]. We undertook a retrospective longitudinal body in 1995-1996, the oldest available serum sample was also study in a cohort of immunocompetent hemophiliacs with longtested to make it possible to date the seroconversion period for term follow-up to investigate this hypothesis.those who tested positive and to detect possible serologic proof of previous infection for those who tested negative. The oldest serum sample that we tested was collected in April 1981. Changes in the serologic profile were analyzed by RIBA using at least 3 serum

No abstract

The physical condition of severe haemophilia and the impact of advances in replacement therapy have been much studied, but little work has been done on patients who developed inhibitors. The 'Statut Orthopédique des Patients Hémophiles avec Inhibiteur' study was conducted in France in order to assess the orthopaedic status and quality of life of such patients, and the cost of their medical management. Fifty haemophiliacs aged 12-63 years with a history of high-responder inhibitors were included. Clinical assessment showed that only 12% of the patients had a nil pain score and 2% a nil clinical score, as per Gilbert scale. The mean clinical score was significantly higher in patients over 35 years of age than in younger ones. However, younger patients appeared to have a more impaired orthopaedic status than young haemophiliacs without inhibitors of similar age in previous published cohorts. Surprisingly, older haemophiliacs tended to have the best mental quality of life, contrasting with their highly impaired orthopaedic condition and physical quality of life. The mean cost of clinical resources consumed during the year preceding enrolment was Euro 268 999, 99% of which was related to clotting factor. Marked between-patient differences in cost were noted. Our study suggests that the management of haemophiliacs with inhibitors should be improved in order to prevent haemophilic arthropathy to an extent similar to that of patients without inhibitors. Cost-benefit assessment of any therapeutic strategy should always be combined with quality-of-life evaluation.