La drépanocytose est aujourd’hui la première maladie génétique en France avec environ 30 000 patients adultes. Une mutation ponctuelle sur le chromosome 11 conduit à la production d’une hémoglobine ...pathologique qui polymérise sous l’effet de facteurs endo-ou exogènes induisant la falciformation des globules rouges à l’origine d’une vasoocclusion artérielle dont une des conséquences est l’oblitération des vaisseaux à destinée osseuse. Les infarctus osseux sont particulièrement douloureux, conduisant les patients aux urgences où l’enjeu est de soulager rapidement les douleurs par l’utilisation de morphine principalement par analgésie autocontrôlée après titration morphinique. Le risque majeur au cours de ces crises vaso-occlusives (CVO) est l’apparition d’un syndrome thoracique aigu (STA) pouvant mettre en jeu le pronostic vital du patient. La spirométrie incitative est un moyen préventif du STA important à instituer dès les urgences. Une antibiothérapie sera mise en place en cas de fièvre chez ces patients aspléniques à risque d’infections à germes encapsulés notamment par le pneumocoque. L’échange transfusionnel est une des pierres angulaires du traitement des CVO ou du STA mais le risque d’accident hémolytique aigu post transfusionnel doit en limiter l’usage à des situations mettant en jeu le pronostic vital ou fonctionnel d’organe. Plusieurs scores clinicobiologiques permettent de décider de l’utilité d’un angioscanner thoracique au cours du STA à la recherche d’une embolie pulmonaire ou pour décider de la pertinence d’une sortie vers une hospitalisation à domicile (Programme DREPADOM). L’utilisation de la morphine en dehors de l’hôpital de façon prolongée doit être prudente en raison du risque d’addiction.
Sickle cell disease is today the first genetic disease in France with approximately 30,000 adult patients. A point mutation on chromosome 11 leads to the production of pathological hemoglobin which polymerizes under the effect of endogenous or exogenous factors inducing the sickling of red blood cells causing arterial vaso-occlusion, one of the consequences of which is the obliteration of vessels destined for bone. Bone infarcts are particularly painful, leading patients to emergencies where the challenge is to quickly and effectively relieve pain by using morphine mainly by self-controlled analgesia after morphine titration. The major risk during these vaso-occlusive crises (VOCs) is the appearance of an acute thoracic syndrome (ATS) which can be life-threatening for the patient. Incentive spirometry is an important preventive means of ATS to be instituted in emergencies. Antibiotic therapy will be implemented in the event of fever in these asplenic patients at risk of infections with encapsulated germs, in particular pneumococcus. Transfusion exchange is one of the cornerstones of the treatment of VOC or ATS, but the risk of acute hemolytic accident post transfusion must limit its use to situations involving the vital or functional prognosis of an organ. Several clinico-biological scores make it possible to decide on the usefulness of a thoracic computed tomography (CT) angiography during the ATS in search of a pulmonary embolism or to decide on the relevance of discharge to home hospitalization (DREPADOM program). Prolonged use of morphine outside the hospital should be cautious because of the risk of addiction.
Les délais d’accès aux soins sont directement associés au pronostic de nombreuses situations et pathologies urgentes telles que l’arrêt cardiaque extrahospitalier, l’accident vasculaire cérébral, ...l’infarctus du myocarde ou le traumatisme grave. Ils représentent ainsi un critère de qualité et d’efficacité du système préhospitalier. Or, les déterminants de l’accessibilité aux soins urgents, donc des délais de prise en charge préhospitalière jusqu’au soin définitif, sont multiples, intriquant notamment des dimensions organisationnelles, géographiques et socioéconomiques, captées par différentes définitions de l’accessibilité aux soins. La mesure de l’accessibilité aux soins urgents est donc complexe et nécessite l’emploi de méthodes spécifiques. Ses déterminants sont sujets à d’importantes disparités territoriales, tant sur le plan national que local, qui conduisent à de fortes inégalités de santé en situation urgente. L’organisation du système de soins préhospitaliers doit ainsi prendre en compte l’ensemble des définitions de l’accessibilité en vie réelle, afin de répondre à des objectifs de performance ajustés aux enjeux particuliers des pathologies traceuses les plus urgentes. Les prochaines évolutions organisationnelles et technologiques en médecine d’urgence devraient permettre de mieux appréhender les déterminants de l’accessibilité à toutes les phases de la prise en charge préhospitalière, vers un rééquilibrage de l’inadéquation entre les besoins réels et l’offre possible de soins urgents.
Delays in access to care are directly associated with the prognosis of many urgent situations and pathologies such as out-of-hospital cardiac arrest, stroke, myocardial infarction, or severe trauma. Thus, they represent a quality and efficiency criterion for the prehospital system. However, the determinants of accessibility to urgent care, and therefore of the delays from prehospital care to definitive care, are multiple, intertwining in particular organizational, geographical, and socioeconomic dimensions, captured by different definitions of accessibility to care. The measurement of accessibility to urgent care is therefore complex and requires the use of specific methods. Its determinants are subject to significant territorial disparities, both nationally and locally, which lead to strong health inequalities in emergency situations. The organization of the prehospital care system must therefore take into account all the definitions of accessibility in real life, in order to meet performance objectives adjusted to the specific challenges of the most urgent pathologies. The next organizational and technological developments in emergency medicine should make it possible to better understand the determinants of accessibility at all phases of prehospital care, toward a rebalancing of the mismatch between real needs and the possible supply of urgent care.
Thrombopénie et grossesse Khellaf, M.; Loustau, V.; Bierling, P. ...
La revue de medecine interne,
August 2012, Volume:
33, Issue:
8
Journal Article
Peer reviewed
La survenue d’une thrombopénie au cours de la grossesse est assez fréquente (∼10 %). Les causes de thrombopénie sont dominées par la thrombopénie gestationnelle (>75 %), qui ne nécessite ni ...exploration ni traitement particulier ; elle apparaît au cours du dernier trimestre de grossesse et se corrige spontanément après l’accouchement. Les autres étiologies sont : le purpura thrombopénique immunologique (PTI) primaire ou associé à d’autres pathologies qui peut apparaître précocement au premier trimestre de la grossesse, les microangiopathies thrombotiques et les syndromes obstétricaux thrombopéniants : éclampsie et le syndrome HELLP (hemolysis, elevated liver enzymes, low platelet count). Le traitement de la pré-éclampsie et du HELLP repose sur des mesures de réanimation symptomatiques et l’extraction fœtale qui sera discutée en fonction du terme et de la sévérité du tableau. Le traitement des microangiopathies fait appel aux mesures de réanimation et aux échanges plasmatiques. Au cours du PTI, aucun traitement spécifique n’est nécessaire pendant la grossesse, sauf en cas de saignement ou de chiffre de plaquettes inférieur à 30×109/L. Il importe de bien préparer l’accouchement qui, sauf contre-indication obstétricale, aura lieu par voie basse. Un chiffre de plaquettes de 50×109/L est requis pour l’accouchement, et de 75×109/L pour une rachianesthésie. Le traitement fait appel à une courte cure de corticoïdes associée aux immunoglobulines dans les formes les plus sévères résistant à la corticothérapie. Il existe un risque de thrombopénie néonatale nécessitant un contrôle de l’hémogramme chez le bébé à la naissance et dans les cinq jours suivants, un traitement du nouveau-né est nécessaire si les plaquettes sont inférieures à 20×109/L.
The occurrence of thrombocytopenia during pregnancy is frequent (about 10%). Etiologies of thrombocytopenia are dominated by the gestational thrombocytopenia (>75%), which requires no exploration and no specific treatment; it usually occurs during the last trimester of pregnancy and corrects itself spontaneously after delivery. Other etiologies are: (1) immune thrombocytopenia (ITP) either primary or associated with other pathologies; ITP may appear early in the first trimester of pregnancy, (2) thrombotic microangiopathy syndromes, and (3) obstetric thrombocytopenia: eclampsia and HELLP syndrome (hemolysis elevated liver enzymes, and low platelet count). Treatment of pre-eclampsia and HELLP syndrome is based on resuscitative measures and symptomatic fetal extraction that will be discussed according to the term and severity of the case. The treatment of microangiopathy is based on resuscitation and plasma exchange. For ITP, no specific action is needed during pregnancy and only symptomatic patients with a platelet count less than 30×109/L must receive a treatment. It is important to prepare the childbirth that can be vaginally except if there is an obstetric contraindication. A platelet count of 50×109/L is required for the delivery, and of 75×109/L in case of spinal anesthesia. Treatment implies a short course of corticosteroids associated with infusion of immunoglobulins in the most severe forms or in case of steroids resistance. There is a risk of neonatal thrombocytopenia requiring a control of the blood count for the baby at birth and within 5 days, newborns have to be treated if the platelet count is less than 20×109/L.
This prospective observational cohort study aimed to explore the clinical features of incident immune thrombocytopenia in adults and predictors of outcome, while determining if a family history of ...autoimmune disorder is a risk factor for immune thrombocytopenia. All adults, 18 years of age or older, recently diagnosed with immune thrombocytopenia were consecutively recruited across 21 hospital centers in France. Data were collected at diagnosis and after 12 months. Predictors of chronicity at 12 months were explored using logistic regression models. The association between family history of autoimmune disorder and the risk of developing immune thrombocytopenia was explored using a conditional logistic regression model after matching each case to 10 controls. One hundred and forty-three patients were included: 63% female, mean age 48 years old (Standard Deviation=19), and 84% presented with bleeding symptoms. Median platelet count was 10×10(9)/L. Initial treatment was required in 82% of patients. After 12 months, only 37% of patients not subject to disease-modifying interventions achieved cure. The sole possible predictor of chronicity at 12 months was a higher platelet count at baseline Odds Ratio 1.03; 95%CI: 1.00, 1.06. No association was found between outcome and any of the following features: age, sex, presence of either bleeding symptoms or antinuclear antibodies at diagnosis. Likewise, family history of autoimmune disorder was not associated with incident immune thrombocytopenia. Immune thrombocytopenia in adults has been shown to progress to a chronic form in the majority of patients. A lower platelet count could be indicative of a more favorable outcome.
Anakinra treatment has been reported to be effective in some patients with systemic-onset juvenile idiopathic arthritis (SoJIA) or adult-onset Still disease (AoSD).
To assess the efficacy and the ...safety of anakinra treatment in SoJIA and AoSD.
SoJIA and AoSD patients were treated with anakinra (1-2 mg/kg/day in children, 100 mg/day in adults); we analysed its effect on fever, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels, numbers of swollen and tender joints, the assessment of disease activity (by physician and parent/patient) and pain (by parent/patient), and American College of Rheumatology (ACR) pediatric core set criteria for JIA activity.
A total of 35 patients were included, 20 with SoJIA and 15 with AoSD. Their mean age (range) at the onset of treatment was 12.4 (3-23) and 38.1 (22-62) years, respectively; disease duration was 7.0 (1-16) and 7.8 (2-27) years, respectively. Active arthritis was present in all cases but one. Of the 20 SoJIA patients, 5 achieved ACR 50% improvement in symptoms (ACR50) response criteria at 6 months. Steroid dose had been decreased by 15% to 78% in 10 cases. A total of 11 of the 15 AoSD patients achieved at least a 50% improvement for all disease markers (mean follow-up: 17.5 (11-27) months). Steroids had been stopped in two cases and the dose was decreased by 45% to 95% in 12 patients. Two patients stopped anakinra due to severe skin reaction, and two patients due to infection: one visceral leishmaniasis and one varicella.
Anakinra was effective in most AoSD patients, but less than half SoJIA patients achieved a marked and sustained improvement.
Romiplostim and eltrombopag, the first thrombopoietic receptor-agonists with demonstrated efficacy against immune thrombocytopenia in prospective controlled studies, were recently authorized in most ...countries for adults with chronic immune thrombocytopenia. So far, no data are available about the potential contribution of switching from romiplostim to eltrombopag or vice versa in terms of efficacy or tolerance. Efficacies and tolerance profiles were evaluated for 46 patients who sequentially received both drugs, switching from one to the other. The reasons for switching were: lack of efficacy for 23 patients, platelet-count fluctuations for 11, side effects for 4, and 8 patients' preferences. For 50-80% of the patients, switching from romiplostim to eltrombopag or eltrombopag to romiplostim effectively impacted the platelet count, with fluctuations disappearing in 54% and side effects resolved in 100%. In 80% of the patients, the 2 thrombopoietic receptor-agonists achieved similar response patterns. Our results confirmed that switching from one thrombopoietic receptor-agonist to the other could be beneficial in clinical practice for patients with severe chronic immune thrombopenia who failed to respond or experienced adverse events to the first. (Clinical Trials.gov identifier: NCT01618734).
Purpose
Previous clinical trials suggested that inhaled nitric oxide (iNO) could have beneficial effects in sickle cell disease (SCD) patients with acute chest syndrome (ACS).
Methods
To determine ...whether iNO reduces treatment failure rate in adult patients with ACS, we conducted a prospective, double-blind, randomized, placebo-controlled clinical trial. iNO (80 ppm,
N
= 50) gas or inhaled nitrogen placebo (
N
= 50) was delivered for 3 days. The primary end point was the number of patients with treatment failure at day 3, defined as any one of the following: (1) death from any cause, (2) need for endotracheal intubation, (3) decrease of PaO
2
/FiO
2
≥ 15 mmHg between days 1 and 3, (4) augmented therapy defined as new transfusion or phlebotomy.
Results
The two groups did not differ in age, gender, genotype, or baseline characteristics and biological parameters. iNO was well tolerated, although a transient decrease in nitric oxide concentration was mandated in one patient. There was no significant difference in the primary end point between the iNO and placebo groups 23 (46 %) and 29 (58 %); odds ratio (OR), 0.8; 95 % CI, 0.54–1.16;
p
= 0.23. A post hoc analysis of the 45 patients with hypoxemia showed that those in the iNO group were less likely to experience treatment failure at day 3 7 (33.3 %) vs 18 (72 %); OR = 0.19; 95 % CI, 0.06–0.68;
p
= 0.009.
Conclusions
iNO did not reduce the rate of treatment failure in adult SCD patients with mild to moderate ACS. Future trials should target more severely ill ACS patients with hypoxemia.
Clinical trial registration
NCT00748423.
Introduction. –
La survenue de néoplasies et de lymphomes au cours du lupus érythémateux systémique (LES) est quelquefois rapportée. Les liens physiopathologiques entre LES et lymphome non hodgkinien ...restent controversés à ce jour. Nous rapportons un cas de lymphome de Burkitt survenu chez une patiente traitée par immunosuppresseurs pour un LES.
Exégèse. –
Une patiente de 38 ans traitée depuis dix ans par immunosuppresseurs pour un LES présente une mononévrite multiple avec une atteinte rapide des nerfs crâniens, sans atteinte extraneurologique. Le liquide céphalorachidien est normal. Le diagnostic est porté grâce à la biopsie ostéomédullaire montrant une infiltration par un lymphome B de type Burkitt.
Conclusion. –
Il s'agit du troisième cas rapporté dans la littérature de lymphome de Burkitt au cours du lupus. Nous résumons les données de la littérature et les hypothèses physiopathologiques concernant l'association LES et lymphome non hodgkinien.
Introduction. –
Neoplasia and lymphoproliferative disorders are sometimes reported in patients with Systemic Lupus Erythematosus (SLE). However, the pathophysiological link between lymphoma and SLE is still matter of debate. We report a new case of Burkitt's lymphoma occurring in a patient treated by immunosuppressive drugs for a SLE.
Case report. –
A 38-year-old woman with a SLE treated for 10 years by immunosuppressive drugs was admitted for a rapid onset of multiple neuritis with cranial nerves palsy, without extraneurological involvement. The cerebrospinal fluid was normal. A bone marrow biopsy revealed a Burkitt's lymphoma.
Conclusion. –
This is the third case reported of Burkitt's lymphoma occurring in SLE. We discuss herein the data of the literature and the possible pathophysiological links between Burkitt's lymphoma and SLE.
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