The French hemovigilance system provides an accurate estimate of the TTBI incidence during a period with diversion and improving skin disinfection but without bacterial detection screening. This tool would be able to evaluate further additional safety procedures like bacterial screening and pathogen reduction technology.

Part of the French clinical experience using the solvent/detergent (S/D) treated VHP von Willebrand factor (vWF) concentrate (LFB, Les Ulis, France) characterized by a high vWF:RCoF specific activity and a low factor VIII (FVIII) content is reported. Since 1989 this concentrate has been routinely used in clinical practice taking into account the vWF:RCoF given by the manufacturer. Seventy-five patients with von Willebrand disease (type 1: 42, 2A: 11, 2B: 5, 2N: 6, 3: 4, acquired: 7) were treated on 99 occasions either to control spontaneous bleedings (15) or to prevent haemorrhagic risks associated with minor (< 5 days of treatment) (48) or major (5 days of treatment) (36) surgery including seven knee or hip-replacements. Forty lots of concentrate were used containing 58 +/- 13 U mL-1 vWF:RCoF with less than 10 units of FVIII per 100 units vWF:RCoF. Patients with type 2N were analysed separately. With the exception of gastro-intestinal bleedings, spontaneous bleedings were generally stopped after few infusions of 40-47 U kg-1 vWF:RCoF. Patients having more than 20 U dL-1 FVIII were treated on 54 surgical occasions with one preoperative infusion (51-55 U kg-1 vWF:RCoF) which allowed an increase in FVIII concentration to a mean level of 67-88 U dL-1. Patients with less than 20 U dL-1 were either treated with two preoperative infusions of vWF, 12 or 24 h apart (11 cases) or received a FVIII injection immediately after the preoperative infusion of vWF (10 cases). During the postoperative period vWF alone (30-35 U kg-1 vWF:RCoF) allowed FVIII to be kept at a mean level of 118-138 U dL-1 not exceeding 180 U dL-1. Patients with type 2N were treated taking in account only their baseline FVIII concentration. No haemorrhagic complications occurred in any of the patients. Thus it was found to be feasible and practical to manage replacement therapy in patients with von Willebrand disease (whatever the type or the circumstances) on the basis of the vWF:RCoF activity of the concentrate.

Between 1970 and 1994, 116 chemical and 90 radioactive synovectomies were performed in 107 patients with severe haemophilia and two with type 3 von Willebrand's disease. The products used were osmic acid (OA) in 100 cases, 90-Yttrium in 35 cases, 186-Rhenium in 48, 169-Erbium in two, hexacetonide triamcinolone in 16 and radioactive gold in five cases. The use of radioactive colloids is not allowed in France in patients under 15 years of age. Twenty-nine patients had more than one synovectomy per joint. All patients were evaluated for 6 months post-synovectomy, using both a clinical and a radiological score. Six months after synovectomy, a good or excellent result was obtained for 81% of the joints treated with isotopes, compared with 44% of those treated with OA, P<0.001. This superiority of isotopes over osmic acid was still observed after 6 months for the 89 joints that were re-evaluated, with follow-up ranging from 1 to 9 years. It was possible to calculate a radiological score in 84 cases. With OA the best results were from the joints with the lowest scores pre-synovectomy (<7). No correlation could be established between the clinical and the radiological scores, due to the small size of the sample. In summary: (1) chemical and radioactive synovectomy are simple and safe procedures for haemophilic arthropathy, (2) in our series, after 6 months the efficacy of isotopic synovectomy was greater than that of chemical synovectomy, and this benefit seems to persist after 6 months, and up to 9 years in the group of patients with longer-term follow-up.

Y. enterocolitica has been identified with increased frequency as a causative agent of posttransfusion septic shock. This nationwide investigation of these cases led to an estimated incidence of one case per 6.5 million RBC units distributed in France. Although rare, this often fatal complication remains nonpreventable worldwide owing to the lack of practical means for screening RBCs before transfusion.

SummaryLiver histology is important for prognosis and treatment strategy in patients with hepatitis C. We report a 10-year experience of transjugular liver biopsy (TJLB) in patients with haemophilia and other congenital bleeding disorders (CBD) in terms of safety, efficiency and therapeutic consequences. TJLB was proposed to patients who were regularly followed for CBD, and were hepatitis C virus (HCV) positive by polymerase chain reaction. Patients with inhibitors or who were human immunodeficiency virus (HIV) positive with CD4 cells <0AE2 · 10 9 /l or with evidence of liver failure were excluded. TJLB was performed during a short hospitalization with factor replacement.

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Background: Transfusion Related Acute Lung Injury (TRALI) is a life-threatening complication of allogenic blood transfusion, manifested typically by a non-cardiogenic pulmonary edema. However, the magnitude of the risk of TRALI remains unknown at this time, all the more so as a variety of other clinically similar respiratory complications can be associated with transfusion. Hemovigilance had been implemented in France since 1994. All adverse effects (AE) regardless of their severity should be notified on a normalized form to AFSSaPS, the French health products safety agency, as required by the French law. Objective : to describe TRALI observed in e-FIT, the French hemovigilance database. Population and methods: AE possibly, probably or definitely associated with transfusion were considered for inclusion. As the item “ TRALI” was explicitly present since 09/01/2001, we screened the database from 07/01/1994 to 09/01/2001 with “ pulmonary edema AND fluid overload excluded” , or with free comments including the words “TRALI ” , “ anti-granulocyte antibodies ”, “ non-cardiogenic pulmonary edema ” , “ white lung syndrome ” . The subsequent AE were considered for inclusion only if TRALI was the final diagnostic. Results: From 07/01/1994 to 06/01/2006, 82,436 AE were registered in e-FIT, corresponding to about 30,000,000 labile blood products (LBP) transfused. 117 AE fulfilled the above criteria (3.9/1,000,000 LBP transfused). There were 59 men and 58 women, age = 0 to 86 (median 58); 34 men and 21 women were 60 years old or more. 107 patients presented with pulmonary symptoms, 93 with dyspnea, 63 with chills and/or hyperthermia and 53 with a typical pulmonary edema. 13 TRALI were fatal, 85 were life-threatening and 19 were benign. 42 patients received only packed red cells, 4 only fresh frozen plasma and 40 only platelet concentrates ; among the 31 remainders, 14 received platelets concentrates associated with an other LBP. Conclusion: Although the initial French database was not adapted to detect TRALI cases until 2001, the dynamics of notification is of interest ; TRALI is more frequent in elderly patients, as noted in other reports ; Platelets concentrates remains the more frequently LBP involved, but packed red cells also contribute to nearly half of the TRALI reported ; Besides the classical life-threatening presentation, a benign form of TRALI seems to exist ; A national working party is implementing new ways to define and investigate TRALI, which will provide more accurate information on TRALI in France.

The aims of the study were to evaluate the impact of a written information about treatment related risks in patient receiving blood derived or recombinant medications. Haemophiliac patients and patients with constitutional or acquired immune deficiencies are concerned by this treatment and these information. Our objectives are to evaluate the efficacy of the written information, the knowledge of the patients about these medications and the psychological, emotional impact if these information. The study is based on questionnaires which specified how the patient treat bleeding episodes, their knowledge about viral safety of blood products, the patient's perception of his or her health status and relationship with the physician. Psychological and emotional status are evaluated with the Hospital Anxiety and Depression Scale. The results show the difficulty to inform patients: if the information generate only limited anxiety in patients with haemophilia or immune deficiencies, we observe that the delivery of a written information got a mediocre effect on overall knowledge. We think that this information must be appropriate for patients and be communicated orally within the patient-physician relationship.