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    Efficacy and Thrombotic Risk of Thrombopoietin Receptor Agonists for Immune Thrombocytopenia Secondary to Systemic Lupus and Antiphospholipid Syndrome: French Experience With 80 Patients

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    International audienceABSTRACT Only few data are available regarding the efficacy and safety of thrombopoietin receptor agonists (TPO‐RAs) for treating systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS)‐associated immune thrombocytopenia (ITP). We retrospectively assessed the efficacy and safety of TPO‐RAs in 80 adults with ITP in three subgroups: (1) 37 patients with definite or incomplete SLE and no antiphospholipid antibodies (APAs), (2) 27 patients with definite or incomplete SLE associated with APAs and no history of arterial or venous thrombotic events (TEs) and (3) 16 patients with APS. In total, 39 (48.8%) patients received eltrombopag, 14 (17.5%) romiplostim and 27 (33.8%) both TPO‐RAs sequentially. The overall response of ITP was 78.8%. In total, 17 (21.3%) patients had 21 TEs (7 venous and 14 arterials, including 3 catastrophic APS); 3 were fatal. The rate of TEs was 8.1% in the SLE or lupus without APAs group, 22.2% in the SLE or lupus with APAs group and 50% in the APS group. In 12 patients, TPO‐RAs were continued after TE onset, combined with an anticoagulant ( n = 12) and/or an antiplatelet agent ( n = 3). Only one TE relapse occurred in the nine patients with TPO‐RAs maintained and combined with an anticoagulant after TE. TPO‐RAs may be effective safe in ITP associated with SLE and no APAs. Their use should be carefully considered in the presence of thrombotic risk factors and/or APA positivity and should be avoided in patients with definite APS. When a TE occurs, risk of relapse is low with maintenance of the TPO‐RA combined with anticoagulation

    Influence of geographical accessibility to specialist and primary care givers on excess mortality of multiple sclerosis patients in France

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    International audienceBackground: Access to healthcare and socioeconomic deprivation are intricately linked. No studies have been led to measure the effect of healthcare accessibility on mortality in patients with MS so far. The objective was to examine the influence of travel time to the expert MS centre and of the accessibility to primary healthcare services on excess mortality in MS.Methods: A retrospective observational cohort study recruited patients from 18 French MS expert centres, with an onset of MS between 1960 and 2015 and a follow-up of up to 30 years. Primary health facility accessibility was measured by the Spatial aCcessibility multiscAlar index. Specialist care accessibility was measured by road travel time to the expert MS centre. Excess death rates (EDR) and excess hazard ratios were studied using additive excess hazard models with multidimensional penalised splines.Results: The study included 33,697 patients. Patients with relapsing-onset MS (R-MS) with a travel time of 40 min had the lowest EDR (Men: 1.2 deaths per 100 person-years (95%CI [0.8;1.8]), women: 0.8 deaths per 100 person-years 95%CI[0.6;1.2]), lower than patients who lived further from the centre. No effect of primary care access was found for patients with R-MS, and no effect of accessibility to primary or specialised care was found for patients with primary progressive MS.Conclusion: This study reveals the impact of travel time to neurologists on excess mortality in patients with R-MS in France. This distance bias association highlights the importance of preventing a potential selection of patients followed in MS expert centres

    Allogeneic Hematopoietic Stem Cell Transplantation for Elderly Acute Lymphoblastic Leukemia Patients: A Registry Study From the Société Francophone de Greffe de Moelle et Thérapie Cellulaire (SFGM-TC).

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    International audienceABSTRACT There are very limited data regarding the outcomes of elderly patients with acute lymphoblastic leukemia (ALL) who undergo allogeneic hematopoietic stem cell transplantation (alloHSCT). A total of 316 ALL patients aged ≥ 60 years who underwent alloHSCT between 2010 to 2022 were identified in the SFGM‐TC registry. The primary objective was to evaluate progression‐free survival (PFS), non‐relapse mortality (NRM), relapse incidence (RI), and graft‐versus‐host disease (GvHD)‐free relapse‐free survival (GRFS), as well as their risk factors. The median age was 63.8 years (range 60–75.8), 49.8% of patients had Philadelphia‐positive B‐ALL (Ph + ALL), and 70.9% were in first complete remission (CR1) at transplantation. The donor was an unrelated donor in 52.1%, a matched related donor (MRD) in 26.3%, and a haplo‐identical donor in 17.7%. Reduced‐intensity conditioning (RIC) was administered to 64.6% of patients, while total body irradiation (TBI) was used in 35.8%. The 3‐year overall survival (OS) was 46% (95% CI 40%–53%). The 3‐year PFS, NRM, RI, and GRFS were 41% (95% CI 35%–48%), 23% (95% CI 18%–28%), 36% (95% CI 31%–42%), and 30% (95% CI 25%–37%), respectively. Multivariable analyses confirmed poorer OS and PFS in patients with advanced disease, with an HR of 1.79 (95% CI 1.22–2.64), p = 0.0032. Additionally, the ALL subtype significantly impacted outcomes, with an HR of 1.99 (95% CI 1.42–2.79) for non‐Ph + ALL. This study suggests that alloHSCT is a viable option for elderly ALL patients, as age itself did not impact outcomes. However, advanced disease and non‐Ph + ALL were associated with significantly worse survival

    Introduction Recommandations pour la pratique Clinique 2021

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    National audienc

    Is systematic deltoid ligament repair necessary in bimalleolar equivalent ankle fractures? A bi-centric case-control study

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    International audienceBackground: Bimalleolar equivalent fractures (BEF) represent a particularly challenging subset of ankle fractures, with ongoing debate among ankle surgeons about the necessity of systematic deltoid ligament repair. The present study aims to address this controversy by investigating the radiological and clinical outcomes of deltoid ligament repair in BEF compared to conservative management. Hypothesis: Systematic deltoid ligament repair in BEF is not necessary. Patients and methods: This bi-centric case-control study included 67 patients from two university hospitals. Patients were divided into two groups: the conservative group (n = 38), and the suture group (n = 29). Inclusion criteria were lateral malleolus fracture with Medial Clear Space (MCS) ≥4 mm, and age ≥18 years. Primary radiological outcomes were MCS and tibio-fibular clear space (TFCS) postoperatively and at 3 months. Clinical outcomes included weight-bearing capacity, time to return to pre-injury activity levels, and subjective sensation of ankle instability. Results: Radiologically, both groups showed significant reduction in MCS and TFCS postoperatively, with no significant difference at the three-month follow-up. Clinically, the conservative group demonstrated significantly higher weight-bearing capacity at 45 days (p = 0.005) and earlier return to pre-injury activity levels (p = 0.0049). Subjective instability was reported less frequently in the conservative group (p = 0.0038). In patients with syndesmotic injuries, no significant differences were observed in radiological or clinical outcomes between the two groups. Conclusion: This study suggests that conservative management of the deltoid ligament in BEF provides comparable radiological outcomes and superior early clinical outcomes compared to surgical repair. The findings support the consideration of conservative treatment for BEF. Further research with larger sample sizes and longer follow-up are necessary to validate these results

    Imaging follow-up of unruptured intracranial aneurysms treated with flow diverter: Insights from a French collaborative survey

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    International audiencePurposeThe absence of standardized guidelines for imaging follow-up of unruptured intracranial aneurysms treated with flow diverters (FD) results in significant variability across centers. This survey aims to provide a comprehensive overview of current imaging follow-up practices in French academic centers.MethodsIn April 2024, a 49-question survey was distributed to interventional neuroradiology centers in France through the trainee-led research collaborative network, Jeunes En Neuroradiologie Interventionnelle-Research Collaborative (JENI-RC). The survey covered follow-up protocols for digital subtraction angiography (DSA), magnetic resonance imaging (MRI), and computed tomography (CT), as well as potential modifications for specific cases.ResultsTwenty interventional neuroradiology centers responded to the survey. Most centers (n = 14, 70 %), conducted a single systematic DSA either at 6 or 12 months post-procedure. In most centers (n = 13, 65 %), a second DSA was scheduled only based on the results of the first, specifically in cases of non-occluded aneurysm, intimal hyperplasia, or in-stent stenosis. MRI follow-up varied, with most centers (n = 12, 60 %) performing three MRIs over five years. Only two centers included CT scans in their protocols.ConclusionThis survey highlights the substantial heterogeneity in imaging follow-up protocols for FD-treated unruptured intracranial aneurysms among French academic centers. The findings underscore the urgent need for standardized guidelines to harmonize practices, optimize patient outcomes, and improve resource utilization. Additionally, innovations in FD technology, advancements in MRI sequences, and the advent of photon-counting CT will likely influence follow-up practices in the near future

    Construire la psychiatrie de précision : pourquoi et comment ?

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    International audiencePrecision psychiatry aims to identify homogeneous subgroups of patients using biomarkers, which could revolutionize current diagnostic frameworks and raise hopes for personalized treatments. Early examples in neuropsychiatry demonstrate the feasibility of this approach, such as the reclassification of dementias in neurology based on biomarkers such as amyloid plaques and tau proteins, and significant advances in neurodevelopmental disorders such as autism, where genetic heterogeneity has reshaped diagnostic perspectives. To achieve this vision, major challenges must be overcome, including the establishment of large transdiagnostic cohorts, the integration of multimodal data such as imaging, genomics, or digital phenotyping, and the application of advanced statistical methods such as clustering, normative modeling and digital twins. While this strategy promises more precise and effective care tailored to individual needs, it also challenges traditional classifications and forces the psychiatric community to rethink its foundational paradigms in light of biological knowledge.La psychiatrie de précision ambitionne d’identifier des sous-groupes homogènes de patients grâce à des biomarqueurs. Elle promet de révolutionner les classifications actuelles et génère l’espoir de personnaliser les traitements. Des exemples en neuropsychiatrie montrent que cette approche est réaliste, qu’il s’agisse de la reclassification des démences en neurologie ou des succès récents dans le champ des troubles du neurodéveloppement. Les défis incluent le développement de grandes cohortes transnosographiques, l’intégration de données multimodales et l’utilisation de méthodes statistiques avancées. Cette stratégie promet des soins adaptés et efficaces, tout en questionnant les classifications établies

    T cell malignancies after CAR T cell therapy in the DESCAR-T registry

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    International audienceThe risk of T cell malignancies after chimeric antigen receptor (CAR) T cell therapy is a concern, although the true incidence remains unclear. Here we analyzed the DESCAR-T registry database, encompassing all pediatric and adult patients with hematologic malignancies who received CAR T cell therapy in France since 1 July 2018. Of the 3,066 patients included (2,536 B cell lymphoma, 162 B cell acute lymphoblastic leukemia (ALL) and 368 multiple myeloma), 1,680 (54.8%) received axicabtagene ciloleucel, 205 (6.7%) brexucabtagene autoleucel, 44 (1.4%) lisocabtagene maraleucel and 769 (25.1%) tisagenlecleucel. All multiple myeloma patients received idecabtagene vicleucel, with none receiving ciltacabtagene autoleucel. After a median follow-up of 12.7 months for B cell lymphoma, 17.7 months for B cell ALL and 6.3 months for multiple myeloma, only one (0.03%) patient developed a T cell malignancy after CAR T infusion. Specifically, the patient was diagnosed with a primary cutaneous CD30+ T cell lymphoproliferative disorder (anaplastic lymphoma kinase-negative) 3 years after receiving tisagenlecleucel therapy for diffuse large B cell lymphoma. This was associated with the integration of a CAR clone into the tumor suppressor gene PLAAT4 (phospholipase A and acyltransferase 4). Thus, the development of this secondary T cell malignancy might be linked to the use of CAR T cell therapy. In conclusion, our findings indicate a very low risk of T cell malignancy after CAR T cell therapy

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