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.

Prevalence and epitope specificity of non-neutralising antibodies in a large cohort of haemophilia A patients without inhibitors.

Antibodies (inhibitors and non-neutralising antibodies [NNA]) directed against factor VIII (FVIII) remain the main iatrogenic complication in haemophilia A (HA) patients. Inhibitors reduce FVIII pro-coagulant properties, whereas NNA are directed against non-functional epitopes. NNA are poorly studied and their prevalence, epitope specificity and physiopathology inadequately defined.

[Factor V inhibitor: case report and literature review].

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.

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.

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).

Impact of choice of treatment for bleeding episodes on inhibitor outcome in patients with mild/moderate hemophilia a and inhibitors.

Patients with mild/moderate hemophilia A (MHA) may develop inhibitors to factor VIII (FVIII). In this condition, FVIII clotting activity (FVIII:C) baseline levels may remain stable for some patients, but may be reduced to less than 0.01 U/mL for others.

Cost related to replacement therapy during hospitalization in haemophiliacs with or without inhibitors: experience of six French haemophilia centres.

Replacement therapy in haemophiliacs has a major economic impact on health establishments. We assessed in this prospective study the cost of clotting factor concentrate therapy for haemophilia A or B patients. We compared the overall costs of treated patients with or without inhibitors.

Use of recombinant factor VIIa (NovoSeven) in patients with Glanzmann thrombasthenia.

Recombinant factor VIIa (rFVIIa; NovoSeven, Novo Nordisk, Bagsvaerd, Denmark) appears effective and relatively safe for the treatment of bleeding and for surgical prophylaxis in patients with Glanzmann thrombasthenia as reported to the International Registry on rFVIIa and Congenital Platelet Disorders. One of the shortcomings of the Registry data is the heterogeneity of treatment protocol, including dosage, number of doses used, duration of treatment before declaration of failure, and mode of rFVIIa administration (bolus v continuous infusion). The data are not yet sufficient to define optimal regimens for various indications such as the type of bleeding or the type of procedures.

[Kinetics of erythropoietin blood levels from D-1 to D-29 in kidney transplantation].

This study of serum erythropoietin levels and reticulocytes counts in the first month after kidney transplantation shows that the erythropoietin peak is efficient only when serum creatinine level at the time of the peak is under 200 mumol/l.

Leukotriene B4 in hemodialysis.

Leukotrienes are eicosanoids arising from arachidonic acid via 5 lipooxygenase, an enzyme essentially present in leukocyte cells. Leukotriene B4 might be an indicator of neutropolymorphonuclear leukocyte activation when there is contact with artificial membranes. The level of plasmatic leukotriene B4 was measured at three different times during the hemodialysis treatment in several patients undergoing dialysis on three different membranes (one cellulosic and two synthetics).

[Antiprothrombinase and myocardial infarction. Apropos of 2 cases].

Among the thrombotic events associated with a circulating anticoagulant of the antiprothrombinase type, myocardial infarction is exceptionally reported, which justifies the presentation of two cases. In both patients, myocardial necrosis occurred some time after the antiprothrombinase was discovered, and there was nothing special in its clinical features. No obvious atherosclerotic lesion and no image suggestive of vasculitis were found at coronary arteriography, which suggested that the antiprothrombinase played a predominant role in the genesis of infarction.

[Action of amsidine on the evolution of lymphocyte cultures and on the structure of human metaphase chromosomes].

Chromosomic alterations can be observed after the effect of a antimitotic amsidine on metaphases of cultured human lymphocytes: gaps, breaks, exchange figures, deletions, rings. Moreover we notice, among these cells, particular lines of big sized lymphoplasmocytoid cells, whose rate is abnormally high when compared to reference cultures.

[Evaluation of the biocompatibility of a new cellulose membrane by studying the complement system and histamine liberation].

This biocompatibility of the new cellulosic membrane hemophane (HE) is compared to that of cuprophane (CU) in ten maintenance hemodialysis (HD) patients dialyzed on the two types of membranes in randomized order, under otherwise similar technical conditions. Total white blood cell (WBC) and differential counts, blood concentrations of C3a, and C3d and histamine are determined at start of dialysis (TO) and 10, 20 and 180 minutes thereafter. HE is distinct from CU in exerting a minor effect on the generation of C3a, a minor drop of leucocytes during the course of dialysis (P less than 0.

[Cytogenetic study of 20 cases of refractory anemia].

The myelodysplastic syndromes are a group of hematological disorders not yet clearly defined. The authors describe the chromosomal aspects of 20 cases observed in the region of Clermont-Ferrand in comparison with the bibliographical data.

Chronic myelogenous leukemia (CML) with translocation (8;22): a new variant.

A case of chronic myelogenous leukemia (CML) in a young woman with a new variant Ph1-translocation--i.e., t(8;22) (q24;q12)--is described.