Introduction. - Inferior vena cava agenesis is a rare congenital anomaly, generally associated with thrombophilic conditions, and a predisposing factor for deep venous thrombosis (DVT), rarely complicated by pulmonary embolism, in a young population with atypical clinical features and frequent absence of risk factors. Case report. - We report the case of a 30-year-old woman who developed a right iliac DVT, initially presenting as a low back pain and complicated by a pulmonary embolism, 8 months after a sleeve gastrectomy. Chest CT angiography revealed abnormalities that led to the diagnosis of inferior vena cava agenesis. Thrombophilic screening showed a heterozygous prothrombin gene mutation G20210A and hyperhomocysteinemia. The patient was treated with rivaroxaban with good results after 2 years of follow-up. Conclusions. - In young patients without risk factors developing a deep venous thrombosis, an inferior vena cava anomaly should be considered. Although no therapeutic consensus has been currently established, inferior vena cava agenesis seems to be associated with a high prevalence of thrombophilic disorders. Screening could be useful, particularly in patients with a thrombotic family history. (C) 2019 SPLF. Published by Elsevier Masson SAS. All rights reserved.

CD64 is a high-affinity leukocyte receptor for the Fc portion of IgG [1]. As CD64 expression on neutrophil cells (PMNs) is upregulated specifically after bacterial stimulation, it could be used to discriminate inflammatory states from bacterial infections [1-3]. The objective was a comparison of the time course of CD64 expression on PMN cells between patients undergoing cardiac surgery with extracorporeal circulation (ECC) with septic patients.

The aim of this study was to assess the CD64 kinetics after normal cardiac surgery, an essential step to define the potential use of CD64 as an early and specific infectious marker in this postoperative setting. CD64 is a high-affinity receptor for the Fc portion of IgG. It is weakly expressed on the polynuclear neutrophils (PMNs) at rest [1,2] but increases specifically after bacterial stimulation. Thus, CD64 analysis is proposed as an early infectious marker.

Endothelial cell activation and injuries are important causes of multiorgan failure. Altered fibrinolysis promotes fibrin deposition and may create microvascular alterations during inflammation. C-reactive protein (CRP) is correlated with an increased risk of MOF [1] and CRP may inhibit fibrinolysis [2]. We aimed to determine whether plasma fibrinolysis is related to the SOFA score and von Willebrand factor (vWF antigen), as a marker of endothelium dysfunction, in critically ill patients at ICU admission.

L-asparaginase is commonly used in the chemotherapy regimens for acute lymphoblastic leukaemia. Its use is associated with thrombotic complications in 1 to 14 % of the cases. The pathogenesis of this complication is still unclear. However, the decrease of antithrombin seems to play an important role. We report a case of a 17-year old man with a acute lymphoblastic leukaemia, who developed a cerebral sinovenous thrombosis due to an acquired deficiency of antithrombin and protein C and S following L-asparaginase chemotherapy. We discuss the use of prophylactic supplements of antithrombin and the value of screening of thrombophilia based on the recent medical literature.

We report the case of a 26-year-old man with a chronic Budd-Chiari syndrome with ascites, caused by a hereditary Protein S deficiency, in a Turkish family with consanguinity.In this family, the father, the two sisters and the young brother suffered from severe venous thrombosis of the limbs, with pulmonary embolism in two of them.Those thrombotic events are caused by a hitherto not reported mutation in the PROS 1 gene on chromosome 3, resulting in a severe familial Protein S deficiency.No other thrombophilic defect was detected in the family, despite extensive investigation.Furthermore, we observe hereditary twenty-nail dystrophy in this family, the two genes probably segregating independently.Prophylaxis is discussed.

Background: Recent reports have identified the apnoea and hypopnoea index (AHI) as an additional independent risk factor for cardiovascular morbidity and mortality. However, several studies reported contradictory results about the association between the serum C-reactive protein (CRP) level and the severity of apnoea. Objective: The purpose of this work is to study this association in patients referred to the sleep laboratory for clinical suspicion of sleep apnoea and presenting a wide range of AHI. Methods: Forty-nine consecutive patients were included in the study. The SigmaStat(R) software package (Jandle Scientific) was used. Multilinear regression analysis was tested using a stepwise backward selection of the explicative variables. The clinical characteristics ( diabetes, hypertension, smoking habits, gender) were treated as dichotomous variables, while all other data ( age, BMI, lipids, white blood cells) were continuous ones; high-sensitivity (hs)-CRP was the dependent variable. Results: In univariate analysis, AHI was correlated to hs-CRP: R = 0.43, p = 0.002. In multivariate analyses, we found an independent association between the AHI, adjusted for classical cardiovascular risk factors, and hs-CRP. Conclusion: In a sample of 49 patients, referred to the sleep laboratory for suspicion of sleep apnoea in routine practice, we observed an independent association between the AHI and hs-CRP. Copyright (C) 2006 S. Karger AG, Basel.

The authors report the case of a 49-year old man in whom an inaugural portal vein thrombosis led to the diagnosis of hereditary hemochromatosis. In this case, the increase in ferritinemia and the T2-weighted MRI hepatic segmental hyposignal were considered as consequences of tissular necrosis while they did probe a real iron overload. Genetic testing, revealing C282Y/H63D compound heterozygoty, provided evidence for a diagnosis of hereditary hemochromatosis. Weekly venesections induced a calculated iron depletion of 3.5 g without occurrence of anemia, further supporting the diagnosis. We suggest that hemochromatosis should be considered in the differential diagnosis of idiopathic portal vein thrombosis when signs of abnormal iron accumulation exist.

In B-cell malignancies, it is generally held that the monoclonal components (MC) are produced by the malignant clones. Genetic relatedness implies the concordant expression of light-chain (LC) isotypes in the MC and at the surface of the malignant lymphocytes. We reviewed a series of 91 B-cell leukaemias, immunophenotyped by flow cytometry in our laboratory. A serum MC had been sought in 75 of these patients, and had been found in 23 (31%). Biclonal serum components were detected in three cases. LC concordance could not be assessed in three cases of surface LC-null lymphocytes. Of the 23 MC studied in 20 patients, light-chains were discordant in 39%, mostly due to kappa MC in lambda leukaemias. The origin of LC discordance remains speculative. It could be due to the emergence of subclones with the same primal VDJ gene rearrangement or, alternatively, to the development of new B-cell clones escaping immune surveillance from deregulated T-cells.

Hypofibrinolysis and oxidative damages seem to be involved in the pathogenesis of cardiovascular disease. In-vitro, native LDL (n-LDL) and oxidised LDL (ox-LDL) impair the fibrinolytic balance of endothelial cells. The presence of myeloperoxidase which allows the formation of hypochlorous acid (HOCL) and chloride ions-has been reported in atherosclerotic lesions together with markers of HOCl-induced protein damage. In the present study, we aimed to analyse the effect of LDL oxidised by myeloperoxidase (Mox-LDL) on the complete fibrinolysis process at the surface of endothelial cells in-vitro. In our experimental model, we observed a delay of fibrin clot lysis at the surface of endothelial cells when they are exposed to the Mox-LDL.

s and Programme: European Society of Anaesthesiologists; 9th Annual Meeting with the Swedish Society of Anaesthesiology; Gothenburg, Sweden, 7-10 April 2001: Transfusion and Haemostasis