[Factor V inhibitor: case report and literature review]. / Anticoagulant acquis anti-facteur V : à propos d'un cas et revue de la littérature.

Acquired inhibitors to factor V are considered rare events and the cause is often unknown. Diagnostic haemostasis assays to correctly assess this phenomenon are not always perfect and reproducible. Various treatments have been attempted but a standardised management of patients is still lacking. We report the case of a patient who developed a factor V inhibitor and we take the opportunity to make an inventory of the latest data.

[Low sensibility to the action of thrombomodulin in cirrhotic patients. Interest of thrombinography]. / Faible sensibilité à l'action de la thrombomoduline chez le patient cirrhotique. Intérêt de la thrombinographie.

Cirrhosis is associated with complex hemostatic modifications. Most coagulation factors, either procoagulants or anticoagulants, are reduced. Conventional coagulation tests (prothrombin time, activated partial thromboplastin time) don't allow to precisely identify the thrombotic risk as they are not sensible to coagulation inhibitors deficiencies. The aim of this study was to evaluate the coagulation in a population of cirrhotic patients using thrombinography. We analyzed the plasma samples from 30 cirrhotic patients (10 Child A, 10 Child B, Child C 10) compared to 10 healthy controls using thrombinography with and without thrombomodulin to sensiblise this test at the activated protein C pathway. The results of endogenous thrombin potential, the main parameter, expressed as a ratio (thrombinography with/without thrombomodulin) were significantly higher in cirrhotic patients (0.69 ± 0.16) than in controls (0.49 ± 0.10) which reflects a low sensibility to the action of thrombomodulin. This resistance increases with the severity of the disease assessed by the Child-Pugh score, demonstrating a potential hypercoagulable state. The results of the thrombinography challenge the dogma that cirrhotic patients are naturally "anticoagulated." These results highlight the potential interest of the thrombinography in the detection and monitoring of hypercoagulability in cirrhotic patient. Increasing hypercoagulability with the severity of the disease seems to be correlated with clinical observations since the occurrence of thrombosis is more common when cirrhosis is at an advanced stage.

A case of infantile de novo primary antiphospholipid syndrome revealed by a neonatal arterial ischemic stroke.

The few cases of antiphospholipid syndrome that have been reported in neonates are believed to have resulted from a transplacental transfer of antiphospholipid antibodies. Here we report on a boy with a neonatal stroke revealing a de novo primary antiphospholipid, the mother being free of antiphospholipid antibodies. Other thrombosis risk factors included primiparity, gestational diabetes, macrosomia, polyglobulia, and lipoprotein(a) >30 mg/dL. Anti-cardiolipin and anti-ß(2)-glycoprotein I persisted more than 2 years. Under aspirin therapy, the child did not exhibit recurrence of thrombotic events or symptoms of autoimmunity after a follow-up of 3 years. Our case indicates that clinicians should consider a second retesting for anticardiolipin antibodies and anti-ß(2)-glycoprotein I antibodies, even when children and mother neonatal tests are negative.

Increase in proinflammatory cytokines in peripheral blood without haemostatic changes after LPS inhalation.

INTRODUCTION: Bronchoalveolar fibrin deposition is a characteristic of various lung disorders including acute lung injury, acute respiratory distress syndrome and sepsis. It is secondary to the activation of coagulation and inhibition of fibrinolysis in the alveolar space, and can be stimulated by lipopolysaccharide (LPS) inhalation. The aim of this study was to determine the relation between compartmental stress in the lung and systemic response after LPS inhalation by measuring haemostatic parameters. PATIENTS AND METHODS: 12 healthy subjects underwent a bronchial challenge test with LPS; sequential dosages were performed for 5 biological markers (Interleukin-6 (IL-6), C-Reactive Protein (CRP), Prothrombin Fragments 1 and 2 (F 1+2), cortisol and Plasminogen Activator Inhibitor 1 (PAI-1) before endotoxin inhalation and 2, 4, 6, 8 and 24 hours afterwards. RESULTS: IL-6 and CRP levels in the peripheral blood were higher after LPS inhalation; there was no activation of coagulation and no increase in PAI-1 level. CONCLUSION: This study confirms that despite systemic release of proinflammatory cytokines, LPS inhalation does not induce systemic haemostatic response to LPS challenge.

Evolution of thrombin formation and fibrinolysis markers, including thrombin-activatable fibrinolysis inhibitor, during severe meningococcemia.

A 17-year-old girl presented with Neisseria meningitidis sepsis, with evidence of disseminated intravascular coagulation. Substitution therapy with both antithrombin and protein C concentrates was initiated, leading to clinical and biological improvement. Sequential dosages were performed for biological markers including thrombin-activatable fibrinolysis inhibitor (TAFI). Substitution therapy with both antithrombin and protein C concentrates led to a clinical and biological improvement. Biological markers showed a decrease in thrombin generation and in plasminogen activator inhibitor 1 (PAI-1) and a return of TAFI to a normal value. Discontinuation of substitutive treatment was marked by a clinical relapse at 24 h, with thrombin generation and increase in PAI-1, while TAFI remained unchanged. This report shows the evolution of hemostasis markers during septic shock and provides new data concerning the effects of a substitutive therapy.