[Diffuse large B-cell lymphoma: a revolutionary treatment based on genetically-modified immune cells called CAR T cells]. / Le lymphome diffus à grandes cellules B : nouveau traitement révolutionnaire par des cellules immunitaires génétiquement modifiées appelées «CART cells¼.

Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma. Firstline immunochemotherapy cures approximatively 60 % of patients. The prognosis of patients with refractory disease or with relapsed disease within the first two years after the end of treatment is highly unfavourable. Since June 2019, a new third-line treatment with CAR T cells (chimeric antigen receptor T cells) seems to completely modify the prognosis of these patients. A significant proportion of long-lasting complete responses is obtained with this revolutionary treatment. Quick specialized intervention is required for the unique side effects of this therapy.

Le lymphome B diffus à grandes cellules (LBDGC) est le type histologique de lymphome non Hodgkinien le plus fréquent. Le traitement de première ligne par immunochimiothérapie ne permet de guérir qu'environ 60 % des patients. Les patients présentant une maladie réfractaire à une première ligne de traitement ou en rechute dans les deux premières années suivant le traitement présentent un mauvais pronostic. Disponible depuis juin 2019, un nouveau traitement de 3ème ligne sous forme d'immunothérapie par CAR T cells (acronyme anglais de «chimeric antigen receptor T cells¼) semble modifier complètement le pronostic de ces patients, avec l'obtention d'une proportion importante de réponses complètes de longue durée. Les effets indésirables spécifiques liés à ce traitement demandent une prise en charge rapide et spécialisée.

Pharyngeal flap in velopharyngeal insufficiency: Proposal of an algorithm in a series of 31 patients.

This study evaluated the results of a reproductible protocol indicating the need for a pharyngeal flap in children with cleft palate and velopharyngeal insufficiency (VPI). A retrospective review of all patients operated for a pharyngeal flap between 2010 and 2019 in our center was conducted. After exclusion of patients with primary VPI or residual fistulas, 31 patients' data were analyzed. Our main outcome measure was the improvement of the Borel Maisonny Classification (BMC) by at least 1 rank. Further analysis was made to evaluate the impact of age, type of cleft, and BMC before surgery on the gain in the velopharyngeal function. Of the 31 patients, success was achieved in 29 (93.5%, p<0.005). There was no significant correlation between age and gain in the velopharyngeal function (p = 0.137). There was no significant correlation between type of cleft and gain in the velopharyngeal function (p = 0.148). There was a significant correlation observed between the starting classification and gain in velopharyngeal function. The gain observed was greater as the initial velopharyngeal function was worse (p = 0.035). The use of an algorithm combining clinical assessment with a standardized classification of the velopharyngeal function proved to be a reliable tool for the indication of surgery in patients with VPI. A close follow up is essential in a multidisciplinary team.

Short-term hearing outcome of malleus removal for surgery: An observational cohort study.

OBJECTIVES: The main goal of the present study was to assess hearing outcome for malleus removal in cholesteatoma surgery compared to a group with malleus conservation. The secondary aim was to compare the auditory involvement of the stapes between the two groups. MATERIAL AND METHODS: A single-center observational study included adult patients operated on by ossiculoplasty for acquired cholesteatoma between 2015 and 2019. Endpoints comprised improvement in pure-tone average air-bone gap (PTA-ABG) and air-bone gap (ABG) at conversational frequencies and, independently, per frequency. 136 patients were included: 95 with conserved malleus (M+) and 41 with malleus removal (M(). Mean time to audiometric follow-up was 9 months in both groups. RESULTS: The PTA-ABG improvement was 4.4±12.6dB for the M+ group and 3.8±13.4dB for the M- group, with no significant significance (P=0.8). Better results (not exceeding 7.5dB) were found for the M+ group at 2 and 8kHz (P=0.3 and P=0.052 respectively). Presence or absence of the stapes did not affect the results in either group. CONCLUSION: Those results suggest a negligible role of the malleus in early hearing outcome of tympanoplasty for cholesteatoma. A slight improvement was observed in the M+ group at 2 and 8kHz, but its interpretation remains uncertain.

Sensorineural hearing loss in patients with vestibular schwannoma correlates with the presence of utricular hydrops as diagnosed on heavily T2-weighted MRI.

PURPOSE: The purpose of this study was to assess whether the volume of the vestibular endolymphatic space correlates with the degree of hearing loss using heavily T2-weighted fast imaging employing steady-state acquisition with cycle phase (FIESTA-C) MRI. MATERIALS AND METHODS: A total of 23 patients with vestibular schwannoma, as diagnosed on typical image findings, who underwent FIESTA-C MRI were included. There were 13 women and 10 men with a mean age of 63.5±9.3 (SD) years (range: 49-88years). Two radiologists independently evaluated the volume of the utricle and saccule. Correlation between tumor volume, vestibular endolymphatic space volume and degree of hearing loss - as evaluated with the levels of pure-tone average and speech recognition threshold - were searched for. RESULTS: The mean saccular, utricular and tumor volumes were 3.17±1.1 (SD) mm3 (range: 1.45-5.7mm3), 14.55±5 (SD) mm3; (range: 6.6-23.9mm3) and 17.4±5.5 (SD) mm3; (range: 8.3-25.4mm3), respectively. There was a moderate correlation between the volume of the utricle and the degree of hearing loss as evaluated with the levels of pure-tone average (rho=0.5; P=0.015) and speech recognition threshold (rho=0.58; P=0.004). There were no significant correlations between saccular and tumor volumes and the degree of hearing loss. CONCLUSION: The volume of the utricle in patients with obstructive vestibular schwannoma moderately correlates with the degree of hearing loss.

Correlation of flexion contractures with upper extremity function and pain for spinal muscular atrophy and congenital myopathy patients.

To ascertain the patients' perception of the consequences of elbow flexion contractures and better understand the circumstances at their inception, we surveyed 405 spinal muscular atrophy and congenital myopathy patients. Diagrams of various elbow angles and questions concerning the effect of elbow contractures on daily activities were part of the survey. Of 108 completed responses, effectively a 24% response rate, 49 reported elbow flexion contractures. Thirteen of the 49 patients reported first noting them during extended periods of inactivity. Elbow flexion contractures greater than 25 degrees were intractable and were reported to hamper 17% (77 of 410) of specifically considered daily activities for the 49 subjects. Twenty-nine of the 49 (59%) subjects with contractures complained of contracture-associated hindrance of at least one daily function. Perceived contracture-associated difficulties increased significantly as a function of contracture severity. Elbow contractures were perceived to be useful by 12% of the respondents. The correlation between contracture severity and upper limb discomfort was also striking. We conclude that elbow flexion contractures are common and perceived to be associated with disability and discomfort for about one-half of spinal muscular atrophy and congenital myopathy patients.