Présentation clinique et modalités thérapeutiques des carcinomes thymiques (à propos de 9 cas)

Le carcinome thymique (CT) est une tumeur médiastinale rare, morphologiquement et biologiquement distincte du thymome. Notre étude rétrospective est basée sur 9 CT diagnostiqués à un stade invasif (stade III et IV de Masaoka). La biopsie chirurgicale a un meilleur rendement diagnostique que la ponction transpariétale. Elle permet la réalisation de techniques immunohistochimiques permettant la différenciation avec d'autres tumeurs médiastinales. Le traitement repose sur l'association de chimiothérapie pré-opératoire permettant une résection complète, gage d'un meilleur pronostic, et d'un traitement adjuvant dont les modalités restent à définir. Le protocole VIP associant cisplatine, étoposide, ifosfamide a permis l'obtention de 4 réponses. Enfin, les travaux de recherche s'orientant vers de nouvelles cibles thérapeutiques comme les récepteurs de facteurs de croissance c-KIT. L'imatinib (inhibiteur du récepteur c-KIT) pourrait ainsi compléter l'arsenal thérapeutiqueAMIENS-BU Santé (800212102) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Apparent absence of Pneumocystis jirovecii in healthy subjects

© 2006 University of Chicago PressInternational audienceWe prospectively investigated 30 healthy subjects with normal CD4+ T cell counts in blood and normal findings of spirometry and chest radiography for the presence of Pneumocystis jirovecii, by performing polymerase chain reaction on sputum specimens. Fifty patients with chronic obstructive pulmonary disease were investigated at the same time in the same manner; this group was used as controls for the diagnosis of pulmonary colonization with P. jirovecii. None of the healthy subjects had positive test results, whereas the fungus was detected in 8 patients with chronic obstructive pulmonary disease. The results suggest that in our region (Amiens, France), P. jirovecii is apparently uncommon in healthy subjects and that this population, therefore, plays a minor role in circulation of the fungus within human communities

Sleep apnea and incident unprovoked venous thromboembolism: data from the Pays de la Loire sleep cohort

International audienceBACKGROUND: Previous studies have reported inconsistent findings regarding the association between obstructive sleep apnea (OSA) and incident venous thromboembolism (VTE). More specifically, the association between OSA and unprovoked VTE was barely evaluated. We aimed to evaluate whether apnea hypopnea index (AHI) and nocturnal hypoxemia markers were associated with unprovoked VTE incidence in patients investigated for OSA. STUDY DESIGN AND METHODS: Data from the Pays de la Loire sleep cohort were linked to the French health administrative data to identify incident unprovoked VTE in patients suspected for OSA and no previous VTE disease. Cox proportional hazards models were used to evaluate the association of unprovoked VTE incidence with AHI and nocturnal hypoxemia markers including the time spend under 90% of saturation (T90), oxygen desaturation index and hypoxic burden (HB), a more specific marker of respiratory events related hypoxia. The impact of Continuous positive airway pressure (CPAP) was evaluated in the subgroup of patients who were proposed the treatment. RESULTS: After a median [inter-quartile range] follow-up of 6.3 [4.3-9.0] years, 104 of 7,355 patients developed unprovoked VTE, for an incidence rate of 10.8 per 1,000 patient-year. In a univariate analysis, T90 and HB predicted incident VTE. In the fully adjusted model, T90 was the only independent predictor (HR 1.06; 95%CI [1.01-1.02]; p=0.02). The CPAP treatment has no significant impact on VTE incidence. INTERPRETATION: Patients with more severe nocturnal hypoxia are more likely to have incident unprovoked VTE

Diagnostic contribution of EBUS in Interstitial Lung Desease (excluding Sarcoidosis)

28th International Congress of the European-Respiratory-Society (ERS), Paris, FRANCE, SEP 15-19, 2018International audienc

Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation

International audienc

Severe ADAMTS13 Deficiency in Adult Idiopathic Thrombotic Microangiopathies Defines a Subset of Patients Characterized by Various Autoimmune Manifestations, Lower Platelet Count, and Mild Renal Involvement

International audienceThe significance of ADAMTS13 deficiency in adult thrombotic microangiopathy (TMA) remains controversial. In an attempt to define the characteristics of adult TMA with severe ADAMTS13 deficiency, we determined 2 groups of patients on the basis of ADAMTS13 activity (undetectable or detectable). Clinical presentation, laboratory values, autoimmune manifestations, and outcome were compared between the groups. Patients were included retrospectively from 12 centers. All fulfilled the diagnosis criteria of TMA. Patients with a history of transplantation, cancer and chemotherapy, and Centers for Disease Control and Prevention (CDC) stage C human immunodeficiency virus (HIV) infection were not included. Forty-six patients were included. Thirty-one patients had an undetectable ADAMTS13 activity (25%. Severe ADAMTS13 deficiency was associated with a plasmatic inhibitor in 17 cases (55%), suggesting an immune-mediated mechanism. Patients with undetectable ADAMTS13 were more frequently of Afro-Caribbean origin than patients with detectable ADAMTS13 activity (48.4% vs 13.3%, respectively; p = 0.03). As opposed to patients with detectable ADAMTS13 activity, patients with severe ADAMTS13 deficiency displayed various autoimmune manifestations that consisted of nondestructive polyarthritis (4 cases) associated in 1 case with malar rash and extramembranous glomerulonephritis, discoid lupus (3 cases), and autoimmune endocrinopathies, Raynaud phenomenon, and sarcoidosis-like disease (1 case each). In patients with severe ADAMTS13 deficiency, antinuclear antibodies, anti-double-stranded DNA antibodies, and anticardiolipin antibodies were positive in 22 (71%) cases, 3 (9.7%) cases, and 1 (3.2%) case, respectively. One patient fulfilled the criteria for the diagnosis of systemic lupus erythematosus. During follow-up, 1 patient with severe ADAMTS13 deficiency developed antinuclear antibodies, and 3 others developed anti-double-stranded DNA antibodies, in association with neurologic manifestations and anticardiolipin antibodies in 1 case. Patients with severe ADAMTS13 deficiency also had a lower platelet count (12 x 10(9)/L; range, 2-69 x 10(9)/L) and less severe renal failure (estimated glomerular filtration rate: 78 mL/min; range, 9-157 mL/min) than patients with detectable ADAMTS13 activity (49.5 x 10(9)/L; range, 6-103 x 10(9)/L; p = 0.0004, and 15.8 mL/min; range, 5.6-80 mL/min; p < 0.0001, respectively). End-stage renal failure occurred in 1 patient with severe ADAMTS13 deficiency and in 3 patients with detectable ADAMTS13 activity (3.2% vs 21.4%, respectively; p = 0.08). Flare-up and relapse episodes and survival were comparable between the groups. Taken together, these data indicate that adult idiopathic thrombotic thrombocytopenic purpura, as defined by severe ADAMTS13 deficiency, may occur preferentially in a particular ethnic group, and is characterized by severe thrombocytopenia, mild renal involvement, and a wide spectrum of autoimmune manifestations that may be completed during follow-up. Indeed, apparently idiopathic thrombotic thrombocytopenic purpura may be considered a specific autoimmune disease