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We compared the characteristics of the 13 patients with CNS involvement (CNS+) with 62 patients without evidence of active CNS disease (CNS-). B Introduction: b Central nervous system (CNS) involvement in patients with refractory or relapsed large B-cell lymphoma (LBCL) is associated with an extremely poor prognosis. Treatment with anti CD19 CAR-T cells is safe and effective in patients with relapsed refractory large B-cell lymphoma with active central nervous system involvement. [Extracted from the article]

Abstract: In this retrospective study, chimeric antigen receptor T cells remained effective in patients with relapsed/refractory large B-cell lymphoma after prior exposure to bispecific antibodies (BsAbs) targeting different antigens. These results are relevant to clinical practice, particularly given the increasing use of BsAbs in earlier treatment lines., (© 2024 American Society of Hematology. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.)

Background and Purpose: Peripheral neuropathy is a frequent complication of brentuximab vedotin (BV), used in CD30+ lymphoma treatment. Classic BV-induced neuropathy (BV-CN) is a mild distal sensory axonal polyneuropathy. Severe BV-induced inflammatory neuropathies (BV-IN) have been described. BV-IN contribute to lymphoma-associated morbidity but might be immunotherapy-responsive. Our primary objective was to evaluate the rate of BV-IN. Our secondary objectives were to determine risk factors and warning signs., Methods: We conducted a retrospective cohort study on all patients treated with BV at our center between April 2014 and September 2021. Clinical, biological, and electrophysiological data were collected. BV-induced neuropathy was defined as the occurrence of neuropathy up to 3 months after BV discontinuation. BV-IN was defined with criteria adapted from European Academy of Neurology/Peripheral Nerve Society 2021 electrodiagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy. Other neuropathies were classified as BV-CN., Results: Among 83 patients, 41 (49%) developed neuropathy: 35 BV-CN and 6 BV-IN. Thus, the rate of BV-IN was 7.2%. Compared to patients with BV-CN, no predisposing factor was identified. However, patients with BV-IN more frequently presented muscle weakness (67% vs. 5.7%, p < 0.05), gait disorders (83% vs. 20%, p < 0.05), or acute or subacute onset (67% vs. 14%, p < 0.05). BV-IN was frequently more severe (Common Terminology Criteria for Adverse Events grade ≥3; 50% vs. 0%, p < 0.05). Four patients were treated with immunotherapy., Conclusions: Brentuximab vedotin-induced neuropathy is an overlooked complication. Based on four easily identifiable "red flags", we provide an algorithm to help non-neurologist physicians that care for BV-treated patients to detect BV-IN. The aim of the algorithm is to decrease the diagnostic and management delay of this disabling neuropathy., (© 2024 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Competing Interests: The authors declare no conflict of interest.

Aims: Predicting mortality in severe AL cardiac amyloidosis is challenging due to elevated biomarker levels and limited thresholds for stratifying severe cardiac damage., Methods and Results: This prospective, observational, cohort study included de novo, confirmed cardiac AL amyloidosis patients at the Henri Mondor National Reference Centre. The goal was to identify predictors of mortality to enhance prognostic stratification and improve informed decision-making regarding therapy. Over the 12-year study period, among the 233 patients included, 133 were NYHA III-IV and 179 Mayo 2004 III. The independent predictors for mortality identified were hsTnT, NT-proBNP, cardiac output, and conjugated bilirubin. A novel prognostic, conditional stratification, Mondor amyloidosis cardiac staging (MACS) was developed with biomarker cut-off values for Stage 1: hsTnT ≤ 107 ng/L and NT-proBNP ≤ 3867 ng/L (n = 77; 33%); for stage 2 NT-proBNP > 3867 ng/L (n = 72; 30%). For stage 3, if troponin >107 ng/L, regardless of NT-proBNP then CB 4 μmol/L, was added (n = 41; 17.5%) and stage 4: CB > 4 μmol/L (n = 43; 18.5%). The median overall survival was 8 months 95% CI [2-24]. At 1 year, 102 (44%) patients died and the Kaplan-Meier median survival with MACS Stage 1 was not reached, while stage 2 was 15.2 months (95% CI [11-18]) and stage 3, 6.6 months (95% CI [1-13]). Notably, among European stage II patients, 17.1%, n = 8 were MACS stage 3 and European stage IIIb 21.4% (n = 23) were MACS stage 4. Importantly, among European stage IIIb patients 42.2% (n = 29) were classified MACS stage 4 and 12.5% n = 9 were only MACS stage 2., Conclusions: The Mondor prognostic staging system, including conjugate bilirubin may significantly improve prognostic stratification for patients with severe cardiac amyloidosis., (© 2024 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: Early cardiac amyloidosis (CA) diagnosis enables patients to access effective treatments for better long-term outcomes, yet it remains under-recognised, misdiagnosed and inadequately managed., Aim: To reduce diagnostic delays, we aimed to describe the epidemiological and clinical characteristics and changes over an 11-year period., Methods: This was a retrospective, observational cohort study of all patients referred to the Henri-Mondor Hospital for suspected CA., Results: Overall, 3194 patients were identified and 3022 were included and analysed. Our patients came from varied ethnic backgrounds, and more than half (55.2%) had confirmed CA. Over 11 years, referrals increased 4.4-fold, mostly from cardiologists. Notably, wild-type transthyretin amyloidosis (ATTRwt) became the predominant diagnosis, with referrals increasing 15-fold from 20 in 2010-2012 to 308 in 2019-2020. The number of amyloid light chain (AL) diagnoses increased, whilst variant transthyretin amyloidosis (ATTRv) numbers remained relatively stable. Concerning disease severity, AL patients presented more frequently with severe cardiac involvement whereas an increasing number of ATTRwt patients presented with National Amyloid Centre stage I (22.0% in 2013-2014 to 45.9% in 2019-2020). Lastly, among patients diagnosed with ATTRv in 2019-2020, 83.9% had ATTR Val122Ile cardiac phenotype., Conclusions: This study shows that increasing cardiologist awareness and referrals have increased CA diagnoses. With improved awareness and non-invasive diagnostic techniques, more patients with ATTRwt with milder disease and more ATTRv Val122Ile mutations are being referred and diagnosed. Although more AL cases are being recognised, patients are diagnosed with severe cardiac involvement., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Background and Aims: Self-reported questionnaires are useful for estimating the health-related quality of life (HR-QoL), impact of interventions, and prognosis. To our knowledge, no HR-QoL questionnaire has been developed for cardiac amyloidosis (CA). This study aimed to validate Amylo-AFFECT-QOL questionnaire to assess HR-QoL and its prognostic value in CA., Methods: A self-reported questionnaire, "Amylo-AFFECT" had been designed and validated for CA symptoms evaluation and screening by physicians. It was adapted here to assess HR-QoL (Amylo-AFFECT-QOL) and its prognostic value in CA. To validate the theoretical model, internal consistency and convergent validity were assessed, particularly correlations between Amylo-AFFECT-QOL and the HR-QoL Minnesota Living Heart Failure (MLHF) questionnaire., Results: Amylo-AFFECT-QOL was completed by 515 patients, 425 of whom (82.5%) had CA. Wild-type and hereditary transthyretin amyloidosis (ATTRwt and ATTRv) and immunoglobulin light-chain amyloidosis (AL) were diagnosed in 47.8, 14.7, and 18.8% of cases, respectively. The best HR-QoL evaluation was obtained with five dimensions: "Heart failure," "Vascular dysautonomia," "Neuropathy," "Ear, gastrointestinal, and urinary dysautonomia," and "Skin or mucosal involvement." The global Amylo-AFFECT-QOL and MLHF scores showed significant positive correlations (rs = 0.72, p < 0.05). Patients with a final diagnosis of CA had a global Amylo-AFFECT-QOL score significantly higher than the control group composed by patients with other diagnoses (22.2 ± 13.6 vs. 16.2 ± 13.8, respectively, p -value < 0.01). According to the Amylo-AFFECT-QOL global results, ATTRv patients' QoL was more affected than AL patients' QoL or ATTRwt patients' QoL. Patients with a higher HR-QoL score had a greater risk of death or heart transplant after 1 year of follow-up (log-rank < 0.01)., Conclusion: Amylo-AFFECT-QOL demonstrates good psychometric properties and is useful for quantifying HR-QoL and estimating CA prognosis. Its use may help to improve overall management of patients with CA., Competing Interests: TD received grants or consultancy fees from Akcea, Alnylam, Pfizer, and GSK. SO received honoraria from Pfizer. BF received consultancy fees from Pfizer. EI received honoraria from Pfizer and Janssen-Cilag. RG received honoraria from SANOFI. AB received consultancy fees from Pfizer, Novartis, Vifor Pharma, and AstraZeneca. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Kharoubi, Bézard, Broussier, Galat, Gounot, Poullot, Molinier-Frenkel, Fanen, Funalot, Itti, Lemonnier, Sing Chadha, Guendouz, Mallet, Zaroui, Audard, Audureau, Le Corvoisier, Hittinger, Planté Bordeneuve, Lefaucheur, Amiot, Bequignon, Bartier, Leroy, Teiger, Oghina and Damy.)

Objectives: COVID-19 patients affected by haematological malignancies have a more severe course of the disease and higher mortality, prompting for effective prophylaxis. The present study aims to evaluate the humoral response after mRNA vaccination as well as the impact of a third vaccine dose in patients with lymphoid malignancies., Methods: We conducted a single-centre study, evaluating the serological responses of mRNA vaccination amongst a cohort of 200 patients affected by lymphoid malignancies after two or three doses using an industrial SARS-CoV-2 serology assay for anti-receptor binding domain (RBD) Spike IgG detection and quantification., Results: Among patients with plasma cell disorders, 59 of 96 (61%) had seroconversion (anti-RBD >50 AU/mL), and recent anti-CD38 therapies were associated with lower serological anti-RBD IgG concentrations (median IgG concentration 137 (IQR 0-512) AU/mL vs. 543 (IQR 35-3496) AU/mL; p < 0.001). Patients with B-cell malignancies had a lower seroconversion rate (20/84, 24%) mainly due to the broad usage of anti-CD20 monoclonal antibodies; only 2 of 53 (4%) patients treated by anti-CD20 antibodies during the last 12 months experienced a seroconversion. A total of 78 patients (44 with plasma cell disorders, 27 with B-cell malignancies, and 7 with other lymphomas) received a third dose of vaccine. The seroconversion rate and antibody concentrations increased significantly, especially in patients with plasma cell disorders, where an increment of anti-RBD IgG concentrations was observed in 31 of 44 (70%) patients, with an anti-RBD concentration median-fold increase of 10.6 (IQR 2.4-25.5). Its benefit in B-cell malignancies is uncertain, with only 2 of 25 (8%) patients having seroconverted after the vaccine booster, without increased median antibody concentration., Discussion: A third mRNA vaccine dose significantly improved humoral responses among patients with plasma cell disorders, whereas the effect was limited among patients with B-cell malignancies., (Copyright © 2022 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved.)

A 12 year old boy with Burkitt's lymphoma developed severe hepatitis with hepatomegaly, subclinical jaundice, and a small rise in body temperature, associated with an important rise in SGPT and fall in prothrombin titres, 6 days after anticancer chemotherapy and 24 hours after halothane anaesthesia. Hepatitis A and B serology remained negative. This hepatic failure explained perhaps the unusually severe vincristine toxicity which gave rise to a polyneuritis with important sequelae. The association of halothane hepatitis with antimitotic drugs appeared particularly dangerous, and halothane should probably be avoided in all patients been given or about to be given anticancer chemotherapy.

We study the chart of a new born with an oesophageal atresia. The weight of baby is two pounds and half and clinic look seems moderate: a gastrostomy and feeding jejunostomy are decided. After anaesthetic induction, intubation is impossible, and the tube knocks just under the vocal cords. Dilatations are no possible, and a straight approach is undertaken to cut stenosis and allow an extended intubation. Immediate and distant post operative series have brought the authors on abstract of their attitude after a review of literature.

A 8 year-old girl was referred with a mucoepidermoid tumor of the bronchus. Surgery was tried before a mediastin left likeness; diagnosis resided in histology of the surgical piece. Reading of medical literature, very slight for children, corroborate difficulties of histologic classification of these tumors, and especially their doubtful forecast.

In work carried out over a period of two years in the department of anesthetics and intensive care of the Saint-Etienne University Hospital, the authors report their experience of neuroleptanalgesia using droperidol-dextromoramide in children in 104 cases. The observations may be divided up into 2 groups: - 1 group of children below the age of 2 years; - 1 group of children from 2 to 10 years. This study showed the side-effects and complications common to both groups, in particular, the extrapyramidal syndrome. Furthermore, the authors present their technique of administration.

A glucid-lipid and protein compound nutriment: Trive 1000 was administered to 53 patients during the first three postoperative days in varying doses: -1.000 ml per day to a first group -1.500 ml per day to a second group. The local tolerance was quite good. However, a few accidents of a general character were noted: they were due to a great extent to a non-observance of the rules of preservation. A study of the glucides, in Sorbitol form, showed a good assimilation of the substratum. Glycemia remaining within normal, no glycosuria or ketonuria could be observed. The assimilation of the proteins seemed on the whole satisfactory. No important rise in the blood urea was noted. Nitrogen balances showed that, thanks to this nutriment, almost half of them were positive during the post-aggressive period. The more important the dose of Trive 1000, the more positive the nitrogen balances were. The administration of lipids did not reveal any significant change in the total lipids, triglycerides or cholesterol. A study of the graphic record of lipid levels showed chylomicrons twice. On the opposite, lipoproteins were hardly modified apart from a significant in pre-beta lipoproteins. The quantitative analysis of amino-acids proved interesting. A comparative study of amino-acids both in blood and urine before and after the administration of Trive 1000 showed very little variations in connection with essential amino-acids. If the proportions in the blood of non-essential amino-acids were comparatively but little modified before and after the treatment, on the oppostie, the urinary clearance rate of the same amino-acids after treatment was far more variable and often quite important as far as certain amino-acids were concerned.