[Acquired von Willebrand syndrome: from diagnosis to treatment]. / Le syndrome de Willebrand acquis: du diagnostic au traitement.

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.

Efficacy and safety of a low-molecular-weight heparin and standard unfractionated heparin for prophylaxis of postoperative venous thromboembolism: European multicenter trial.

A randomized, double-blind multicenter trial was performed to compare the safety and efficacy of a new low-molecular-weight heparin (LMWH) (LU 47311, Clivarine) and standard unfractionated heparin for the prophylaxis of postoperative venous thromboembolism. Altogether 1351 patients scheduled to undergo abdominal surgery were included. Main outcome measures included the incidence of thromboembolic events (deep vein thrombosis, pulmonary embolism, or both) and bleeding complications, including wound hematoma. A total of 655 patients received 1750 anti-Xa IU of LMWH plus a placebo injection daily; 677 patients received 5000 IU of unfractionated heparin (UFH) twice a day. Both drugs were found to be equally effective, as 4.7% of patients in the LMWH group and 4.3% in the UFH group developed postoperative thromboembolic complications. However, the incidence of bleeding complications was significantly reduced in the LMWH group: 55 (8.3%) patients in the LMWH group and 80 (11.8%) in the UFH group developed bleeding complications, a relative risk (RR) of 0.70 (95% CI 0.51-0.97;p = 0.03); wound hematoma occurred in 29 (4.4%) of the LMWH group compared with 55 (7.7%) in those in the UFH group for an RR of 0.57 (95% CI 0.37-0.88;p = 0.01). This study confirmed that a very low dose of 1750 anti-Xa IU daily of this new LMWH is as effective as 10,000 IU of UFH for preventing postoperative deep vein thrombosis. At this dose its administration is associated with a significant reduction in the risk of bleeding including wound hematoma.

[Prospective etiologic study of 128 patients with ambulatory dep vein thrombosis]. / Etude étiologique prospective de 128 patients avec une thrombose veineuse profonde survenue en ambulatoire.

Etiology of deep vein thrombosis in ambulant patients (DVTA = TVPA in text) varies: cancer, blood disease, infectious focus, dysimmunity syndrome, dysglobulinemia, extrinsic compression, metabolic disorder, anomaly of hemostasis. A prospective study was carried out between June 1988 and September 1989 by angiologists in 5 regions of France to evaluate the diagnostic rentability of an epidemiologic survey and to determine possible distinctive characters of DVTA. The survey was comprised of a questionnaire, a full clinical examination and screening tests: chest x-ray, abdominopelvic ultrasound imaging, a-uro/gynecologic examination, full blood count, serum iron, ferritin, uric acid, triglycerides, cholesterol, protein electrophoresis, antinuclear antibodies, circulating anticoagulant, hemostasis factors and liver function tests. The study included 128 patients, mean age 60 +/- 16 years with a DVTA developing without a previous immobilization. The usual predominance in the left leg was not observed. The etiology was identified in 33 cases, including 20 (15.6%) as a result of the screening tests: anomalies of hemostasis (8), blood diseases (3), dysimmunity syndromes (4), extrinsic compression (3), cancer metastasis (1) and hypertriglyceridemia in a diabetic (1). The screening procedure was of no greater value in the absence of a triggering or predisposing factor, on the contrary. An anomaly of hemostasis was detected more frequently in the presence of local or regional triggering factors in the men (4 out of 4) and in the women on the pill (4 out of 4). The number of cancers discovered following screening (2%) was smaller than that expected according to the literature (10%).(ABSTRACT TRUNCATED AT 250 WORDS)

[Activated thromboplastin time, plasma heparin and plasma fibrinogen using the Fibrintimer 10]. / Temps de céphaline activé, héparinémie et fibrinogène plasmatique sur Fibrintimer 10.

After a thorough study, and having chosen the machine that is to equip an average sized haemostasis laboratory, the installation then requires certain steps (the reorganisation of work, better adapted reagents, a change of normality, a fine tuning of the technics, the time to get used to the machine). In this perspective, this work describes the regulation of the technic to determine the heparin activity on the Fibrintimer 10 (F 10) by chronometry (thrombin clotting time with variable concentration), a study of the repeatability and reproducibility of activated partial thromboplastin time (APTT), plasma heparin (HEP) and fibrinogen on the Fibrintimer 10, a study of the correlation between the results we got with the F 10 and the thermostat water-bath for the APTT and the HEP and between those we got on the F 10 and the fibrometer for the fibrinogen. The F 10 has allowed us to save more time whilst keeping the same technics and reagents. The determination of the heparin activity, by thrombin clotting time with variable concentration, gives us a method which is quick, cheap and useful for the following-up of the patients undergoing a treatment with standard heparin. The reproducibility and repeatability prove to be good for the 3 tests in our operatory conditions as well as the correlations between the results we get with both methods.