Background Vitamin K Antagonists are the most used anticoagulants in the treatment of thrombotic diseases and their misuse is an important source of medicines-related illness. Purpose To study the effect of targeted information on patients’ knowledge of their VKA treatment. Materials and Methods The study took place between 05/07/2012 and 09/07/2012 as described below: An assessment grid including 18 questions grouped in 4 items was made by pharmacists and checked by the ‘haemostasis-coagulation’ group. It included a) general knowledge, b) meaning of the INR test, c) drug and food interactions and d) signs of bleeding. The students were trained (by pharmacists) and empowered (by specialist members of the ‘haemostasis-coagulation’ group) The pharmacy students evaluated patient knowledge with the grid (T0) The answers were analysed in order to highlight points where knowledge was lacking Targeted therapeutic information was supplied on the deficient points Patients were re-evaluated with the same grid before discharge (T1). Results 73 patients (27 males/46 females) were evaluated and received therapeutic information. The mean age was 66 years. 57% of responses were adequate with 69%, 55%, 37% and 47% of correct answers for items a, b, c and d respectively. 41 (56%) of the 73 patients were re-evaluated before discharge. In this group of patients, 50% answers were correct initially and 78% after education. An improvement of the knowledge was observed for all items with 73% vs. 32%, 66% vs. 30%, 83% vs. 18% and 72% vs. 27% of correct answers at T1 vs. T0 for items a, b, c and d, respectively. Conclusions The improvement in patients’ knowledge of their VKA treatment shows the benefit of this approach based on the patients being educated by empowered pharmacy students. That’s why this process should be extended to other units with VKA patients. Reference VKA, therapeutic information, patients’ knowledge. No conflict of interest.

OBJECTIVE: To assess the rate of early (first trimester) and late (second and third trimester) fetal loss in women who are factor V Leiden homozygous.METHODS: Between December 1995 and February 2007, consecutive, unrelated white women who were factor V Leiden homozygous and who had been pregnant at least once were recruited from 10 French hemostasis units. For reasons of comparison, we included women who were factor V Leiden heterozygous and a group of noncarriers. The frequency of early and late fetal loss was assessed retrospectively and compared among the three groups. The effect of concomitant thrombophilic abnormalities was evaluated. The overall pregnancy outcome was reported.RESULTS: We analyzed 240 thromboprophylaxis-free pregnancies in 95 women who were factor V Leiden homozygous, 425 in 195 women who were factor V Leiden heterozygous, and 182 in 73 women who were noncarriers. The risk of late fetal loss was higher in women who were homozygous (13/95,13.7%) compared with those who were noncarriers (1/73, 1.4%, odds ratio 11.41, 95% confidence interval 1.46-89.46, P=.002), whereas it was similar in women who were heterozygous and in noncarriers (6/195, 3.1% compared with 1/73, 1.4%, P=.68). The percentage of women with early fetal loss was similar in the three groups (P=.81). The live-birth rate was 80%, 84%, and 85%, respectively, for women who where homozygous, heterozygous, and noncarriers (P=.88).CONCLUSION: The factor V Leiden homozygous genotype increases the risk of late fetal loss. However, the overall likelihood of a positive outcome is high in our series of women who were homozygous. (Obstet Gynecol 2009;114:1249-53)

Acquired von Willebrand syndrome is a rare bleeding disorder, which has been related in various diseases including lymphoproliferative disorders or autoimmune diseases. Its diagnosis is an important step before treatment of patients and particularly in case of bleeding. We report four cases from Caen Hemophilia Treatment Center, diagnosed and treated from 1999 to 2008. Mucocutaneous bleeds in every case were the same as in hereditary von Willebrand disease. All patients had no personal or family history of bleeding. Phenotype was identified as type 2 von Willebrand disease with a loss of high molecular weight multimers. Anti-von Willebrand factor inhibitor screening was positive for three patients. The etiological diagnosis was one chronic lymphocytic leukaemia, two monoclonal gammapathies of undetermined significance (MGUS) and one undetermined case. The management of patients need two stages: first infusions of factor von Willebrand/factor VIII concentrates to stop bleeds, then treatment of the underlying disease such as chemotherapy, corticotherapy and treatment with high doses of polyvalents immunoglobulins. In every case, treatment was effective and improved patient's quality of life.

Abnormalities of the protein C natural anticoagulant system, i.e. protein C (PC) and protein S (PS) deficiency, and factor V (FV) Leiden-related activated (A) PC-resistance are among the most frequent risk factors for thrombosis as they could be demonstrated in up to 30% of Caucasian patients with thrombophilia [ 1 De Stefano V Finazzi G Mannucci P.M Inherited thrombophilia: pathogenesis, clinical syndromes, and management. Blood. 1996; 87: 3531-3544 PubMed Google Scholar , 2 Lane D.A Mannucci P.M Bauer K.A Bertina R.M Bochkov N.P Boulyjenkov V et al. Inherited thrombophilia. Part 1. Thromb. Haemost. 1996; 76: 651-662 PubMed Google Scholar , 3 Seligsohn U Lubetsky A Genetic susceptibility to venous thrombosis. N. Engl. J. Med. 2001; 344: 1222-1231 Crossref PubMed Scopus (738) Google Scholar ]. So far, screening for these biological abnormalities has been based on the measurement of PC, PS and APC-resistance and/or identification of the causative G1691A mutation for the FV Leiden [ 1 De Stefano V Finazzi G Mannucci P.M Inherited thrombophilia: pathogenesis, clinical syndromes, and management. Blood. 1996; 87: 3531-3544 PubMed Google Scholar , 2 Lane D.A Mannucci P.M Bauer K.A Bertina R.M Bochkov N.P Boulyjenkov V et al. Inherited thrombophilia. Part 1. Thromb. Haemost. 1996; 76: 651-662 PubMed Google Scholar , 3 Seligsohn U Lubetsky A Genetic susceptibility to venous thrombosis. N. Engl. J. Med. 2001; 344: 1222-1231 Crossref PubMed Scopus (738) Google Scholar , 4 Lane D.A Mannucci P.M Bauer K.A Bertina R.M Bochkov N.P Boulyjenkov V et al. Inherited thrombophilia. Part 2. Thromb. Haemost. 1996; 76: 824-834 PubMed Google Scholar ]. The absence of any abnormality in most (>70%) of the patients highlighted the potential usefulness of simple assays, such as the ProC® Global, for global screening of all these abnormalities. This would rationalize the use of the corresponding expensive individual specific assays to only those samples with an abnormal result to the global assay. A generation of such assays has recently been proposed, based on the activation of endogenous PC by various activators [ 5 Duchemin J Pittet J.L Tartary M et al. A new assay based on thrombin generation inhibition to detect both protein C and protein S deficiencies in plasma. Thromb. Haemost. 1994; 71: 331-338 PubMed Google Scholar , 6 Kraus M Noah M Fickenscher K The PCAT—a simple screening assay for assessing the functionality of the protein C anticoagulant pathway. Thromb. Res. 1995; 79: 217-222 Abstract Full Text PDF PubMed Scopus (61) Google Scholar , 7 Denson K.W.E Haddon M.E Reed S.V Davidson S Littlewood T.J A more discriminating test for APC resistance and a possible screening test to include protein C and protein S. Thromb. Res. 1996; 81: 151-156 Abstract Full Text PDF PubMed Scopus (26) Google Scholar , 8 Robert A Eschwege V Hameg H Drouet L Aillaud M.F Anticoagulant response to Agkistrodon Contortrix Venom (ACV) test: a new global test to screen for defects in the anticoagulant protein C pathway. Thromb. Haemost. 1996; 75: 562-566 PubMed Google Scholar , 9 Preda L Simioni P Legnani C et al. A new global test for the evaluation of the protein C-protein S system. Blood Coagul. Fibrinolysis. 1996; 7: 465-469 Crossref PubMed Scopus (27) Google Scholar , 10 Gable P.S Le D.T McGehee W Rapaport S.I A Protac-based screening test for activated protein C-resistant factor Va and other defects of the protein C anticoagulant pathway. Blood Coagul. Fibrinolysis. 1997; 8: 327-335 Crossref PubMed Scopus (21) Google Scholar , 11 Haas F.J.L.M van Sterkenburg-Kamp B.M Scheepers H.A.M.M A protein C pathway (PCP) screening test for the detection of APC resistance and protein C or S deficiencies. Semin. Thromb. Hemost. 1998; 24: 355-362 Crossref PubMed Scopus (22) Google Scholar ]. Most of these assays are based on the ability of endogenous APC, generated by activation of PC by an extract from Agkistrodon contortrix contortrix venom, to prolong a given clotting time, such as the activated partial thromboplastin time (APTT) [ 6 Kraus M Noah M Fickenscher K The PCAT—a simple screening assay for assessing the functionality of the protein C anticoagulant pathway. Thromb. Res. 1995; 79: 217-222 Abstract Full Text PDF PubMed Scopus (61) Google Scholar , 7 Denson K.W.E Haddon M.E Reed S.V Davidson S Littlewood T.J A more discriminating test for APC resistance and a possible screening test to include protein C and protein S. Thromb. Res. 1996; 81: 151-156 Abstract Full Text PDF PubMed Scopus (26) Google Scholar , 8 Robert A Eschwege V Hameg H Drouet L Aillaud M.F Anticoagulant response to Agkistrodon Contortrix Venom (ACV) test: a new global test to screen for defects in the anticoagulant protein C pathway. Thromb. Haemost. 1996; 75: 562-566 PubMed Google Scholar ], the prothrombin time [ 9 Preda L Simioni P Legnani C et al. A new global test for the evaluation of the protein C-protein S system. Blood Coagul. Fibrinolysis. 1996; 7: 465-469 Crossref PubMed Scopus (27) Google Scholar , 10 Gable P.S Le D.T McGehee W Rapaport S.I A Protac-based screening test for activated protein C-resistant factor Va and other defects of the protein C anticoagulant pathway. Blood Coagul. Fibrinolysis. 1997; 8: 327-335 Crossref PubMed Scopus (21) Google Scholar ], or the dilute Russell viper venom time [ [11] Haas F.J.L.M van Sterkenburg-Kamp B.M Scheepers H.A.M.M A protein C pathway (PCP) screening test for the detection of APC resistance and protein C or S deficiencies. Semin. Thromb. Hemost. 1998; 24: 355-362 Crossref PubMed Scopus (22) Google Scholar ]. The ProC Global assay is the first commercially available APTT-based clotting assay, which was designed to assess the functionality of the PC anticoagulant pathway [ [6] Kraus M Noah M Fickenscher K The PCAT—a simple screening assay for assessing the functionality of the protein C anticoagulant pathway. Thromb. Res. 1995; 79: 217-222 Abstract Full Text PDF PubMed Scopus (61) Google Scholar ]. As previously reported for other assays [ 7 Denson K.W.E Haddon M.E Reed S.V Davidson S Littlewood T.J A more discriminating test for APC resistance and a possible screening test to include protein C and protein S. Thromb. Res. 1996; 81: 151-156 Abstract Full Text PDF PubMed Scopus (26) Google Scholar , 8 Robert A Eschwege V Hameg H Drouet L Aillaud M.F Anticoagulant response to Agkistrodon Contortrix Venom (ACV) test: a new global test to screen for defects in the anticoagulant protein C pathway. Thromb. Haemost. 1996; 75: 562-566 PubMed Google Scholar , 9 Preda L Simioni P Legnani C et al. A new global test for the evaluation of the protein C-protein S system. Blood Coagul. Fibrinolysis. 1996; 7: 465-469 Crossref PubMed Scopus (27) Google Scholar , 10 Gable P.S Le D.T McGehee W Rapaport S.I A Protac-based screening test for activated protein C-resistant factor Va and other defects of the protein C anticoagulant pathway. Blood Coagul. Fibrinolysis. 1997; 8: 327-335 Crossref PubMed Scopus (21) Google Scholar , 11 Haas F.J.L.M van Sterkenburg-Kamp B.M Scheepers H.A.M.M A protein C pathway (PCP) screening test for the detection of APC resistance and protein C or S deficiencies. Semin. Thromb. Hemost. 1998; 24: 355-362 Crossref PubMed Scopus (22) Google Scholar , 12 Toulon P Perez P Screening for risk factors for thrombosis using a new generation of assays developed to evaluate the functionality of the protein C anticoagulant pathway. Hematol. Oncol. Clin. North Am. 2000; 14: 379-389 Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar ], its performance has been evaluated in single-site studies using a unique instrument [ 12 Toulon P Perez P Screening for risk factors for thrombosis using a new generation of assays developed to evaluate the functionality of the protein C anticoagulant pathway. Hematol. Oncol. Clin. North Am. 2000; 14: 379-389 Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar , 13 Dati F Hafner G Erbes H et al. ProC Global: the first functional screening assay for the complete protein C pathway. Clin. Chem. 1997; 43: 1719-1723 PubMed Google Scholar , 14 Ruzicka K Kapiotis S Quehenberger P et al. Evaluation of a new screening assay ProC Global for identification of defects in the protein C/protein S anticoagulant pathway. Thromb. Res. 1997; 87: 501-510 Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar , 15 Toulon P Perez P Demay Y Dornon O Adda R ProC Global, a new assay to evaluate the functionality of the protein C anticoagulant pathway. Preliminary results in 223 consecutive patients with a history of venous thromboembolism. Fibrinolysis Proteolysis. 1998; 12: 347-352 Crossref Scopus (11) Google Scholar , 16 Tripodi A Akhavan S Asti D Faioni E.M Mannucci P.M Laboratory screening of thrombophilia. Evaluation of the diagnostic efficacy of a global test to detect congenital deficiencies of the protein C anticoagulant pathway. Blood Coagul. Fibrinolysis. 1998; 9: 485-489 Crossref PubMed Scopus (27) Google Scholar , 17 Gemmati D Serino M.L Scapoli G.L A modified functional global test to measure protein C, protein S activities and the activated protein C-resistance phenotype. Thromb. Res. 1998; 92: 141-148 Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar , 18 Sillero P.L Fernandez de Velasco J Loscertales J Espinoza J Soto C Tomas J.F ProC Global: an automated screening test for factor V Leiden and prothrombin mutation 20210 G to A detection. Thromb. Res. 2001; 101: 215-216 Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar , 19 Rimmer J.E Cooper P.C Brookfield C.J Preston F.E Makris M Evaluation of a global assay for the investigation of the protein C anticoagulant pathway. Clin. Lab. Haem. 2000; 22: 351-354 Crossref PubMed Scopus (6) Google Scholar , 20 Gemmati D Serino M.L Tognazzo S Ongaro A Moratelli S Gilli G et al. The reduced sensitivity of the ProC Global test in protein S deficient subjects reflects a reduction in the associated thrombotic risk. Blood Coagul. Fibrinolysis. 2001; 12: 691-697 Crossref PubMed Scopus (11) Google Scholar ], except for an European multicenter trial which included five centers using two different brands of instruments [ [21] Toulon P Halbmeyer W.M Hafner G Schmitt Y Randgard B Odpadlik M et al. Screening for abnormalities of the protein C anticoagulant pathway using the ProC Global assay. Results of a European multicenter trial. Blood Coagul. Fibrinolysis. 2000; 11: 447-454 Crossref PubMed Scopus (31) Google Scholar ]. Besides some heterogeneity in the expression of the results (ratio or normalized ratio), analysis of the already published data clearly demonstrated significant differences not only in the cut-off level chosen by the authors, but also in the sensitivity and/or the specificity of the assay for PC pathway abnormalities which could be linked to the type of instrument [ 12 Toulon P Perez P Screening for risk factors for thrombosis using a new generation of assays developed to evaluate the functionality of the protein C anticoagulant pathway. Hematol. Oncol. Clin. North Am. 2000; 14: 379-389 Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar , 13 Dati F Hafner G Erbes H et al. ProC Global: the first functional screening assay for the complete protein C pathway. Clin. Chem. 1997; 43: 1719-1723 PubMed Google Scholar , 14 Ruzicka K Kapiotis S Quehenberger P et al. Evaluation of a new screening assay ProC Global for identification of defects in the protein C/protein S anticoagulant pathway. Thromb. Res. 1997; 87: 501-510 Abstract Full Text Full Text PDF PubMed Scopus (26) Google Scholar , 15 Toulon P Perez P Demay Y Dornon O Adda R ProC Global, a new assay to evaluate the functionality of the protein C anticoagulant pathway. Preliminary results in 223 consecutive patients with a history of venous thromboembolism. Fibrinolysis Proteolysis. 1998; 12: 347-352 Crossref Scopus (11) Google Scholar , 16 Tripodi A Akhavan S Asti D Faioni E.M Mannucci P.M Laboratory screening of thrombophilia. Evaluation of the diagnostic efficacy of a global test to detect congenital deficiencies of the protein C anticoagulant pathway. Blood Coagul. Fibrinolysis. 1998; 9: 485-489 Crossref PubMed Scopus (27) Google Scholar , 17 Gemmati D Serino M.L Scapoli G.L A modified functional global test to measure protein C, protein S activities and the activated protein C-resistance phenotype. Thromb. Res. 1998; 92: 141-148 Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar , 18 Sillero P.L Fernandez de Velasco J Loscertales J Espinoza J Soto C Tomas J.F ProC Global: an automated screening test for factor V Leiden and prothrombin mutation 20210 G to A detection. Thromb. Res. 2001; 101: 215-216 Abstract Full Text Full Text PDF PubMed Scopus (6) Google Scholar , 19 Rimmer J.E Cooper P.C Brookfield C.J Preston F.E Makris M Evaluation of a global assay for the investigation of the protein C anticoagulant pathway. Clin. Lab. Haem. 2000; 22: 351-354 Crossref PubMed Scopus (6) Google Scholar , 20 Gemmati D Serino M.L Tognazzo S Ongaro A Moratelli S Gilli G et al. The reduced sensitivity of the ProC Global test in protein S deficient subjects reflects a reduction in the associated thrombotic risk. Blood Coagul. Fibrinolysis. 2001; 12: 691-697 Crossref PubMed Scopus (11) Google Scholar , 21 Toulon P Halbmeyer W.M Hafner G Schmitt Y Randgard B Odpadlik M et al. Screening for abnormalities of the protein C anticoagulant pathway using the ProC Global assay. Results of a European multicenter trial. Blood Coagul. Fibrinolysis. 2000; 11: 447-454 Crossref PubMed Scopus (31) Google Scholar ]. The aim of the present multicenter study was to evaluate the potential influence of several instruments on the results of the ProC Global assay. For that purpose, two lyophilized control human plasmas and frozen aliquots from identical plasmas, obtained from both carriers and non-carriers of the FV Leiden mutation, were analyzed in five laboratories on 11 coagulation analyzers.

Factor V Leiden is the most common risk factor in thrombophilia. However, whether homozygosity confers a higher risk than heterozygosity remains questionable. The first phase of the Procare Study was to collect clinical and biological data from individuals carrying the R506Q mutation. To date, 566 individuals have been included in 23 French centres. Data from 85 homozygotes were compared with those of 481 heterozygotes. Mean age at first thrombosis was earlier for homozygous females versus heterozygous females (P=0.02) or versus homozygous males (P<0.005). Thrombosis occurred more frequently spontaneously in homozygotes than in heterozygotes [odds ratio, 4.1; 95% confidence interval (CI), 2.27-7.42]. Idiopathic events occurred earlier in homozygous females compared with males (P<0.001). In our homozygous population, the 20210A allele of the prothrombin gene may constitute one associated risk condition. Recurrence after a first event was statistically more frequent in homozygotes than in heterozygotes (odds ratio, 1.95; 95% CI, 1.08-3.53). Therefore, homozygosity seems to confer a higher risk of thrombosis than heterozygosity, particularly in women. Blood Coagul Fibrinolysis 11:511-518 (C) 2000 Lippincott Williams & Wilkins.

Nous rapportons le cas d'une femme de 58 ans chez qui une thrombopenie tardive secondaire a l'administration d'une heparine de bas poids moleculaire s'est compliquee d'une thrombose veineuse cerebrale avec infarctus hemorragiques parietaux bilateraux, L'evolution a ete progressivement favorable apres le remplacement de l'heparine de bas poids moleculaire par un anticoagulant oral.

Introduction. – Les aspects usuels de l'atteinte neurologique au cours de la maladie de Behçet consistent en la méningoencéphalite et la thrombophlébite cérébrale. L'atteinte médullaire reste rare surtout comme localisation unique.Exégèse. – Nous rapportons trois cas de myélopathie aiguë compliquant une maladie de Behçet visualisée par imagerie par résonance magnétique, et révélatrice dans deux cas de la maladie de Behçet. Les trois cas sont des myélites transverses dont deux sont des formes juvéniles de neuro-Behçet et deux sont des méningomyélites.Conclusion. – L'imagerie par résonance magnétique permet aisément de faire le diagnostic de myélopathie au cours de la maladie de Behçet mais également de suivre l'évolution sous traitement. Devant toute myélopathie, il faut rechercher des signes pouvant faire évoquer une maladie de Behçet, en particulier en milieu méditerranéen.Introduction. – Neurological manifestations in Behçet's disease usually consist of meningoencephalitis and cerebral thrombophlebitis. Myelitis is rare, especially when it is the only neurological manifestation.Exegesis. – The authors report three cases of acute myelitis complicating Behçet's disease demonstrated by magnetic resonance imaging. In two cases, Behçet's disease was revealed by myelitis. The three cases were transverse myelitis, two of which were juvenile Behçet's disease and two were meningomyelitis.Conclusion. – Magnetic resonance imaging can help guide diagnosis of myelitis in Behçet's disease. It can also be useful to assess the results of the treatment. Clinical symptoms of Behçet's disease must be searched for in the case of myelitis, especially for patients from Mediterranean countries.