Neither the patient nor the physician could see anything: Atypical Bing-Neel syndrome

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Neither the patient nor the physician could see anything: Atypical Bing-Neel syndrome

International audienc

Immunochemotherapy versus rituximab in anti-MAG neuropathy: a report of 64 patients

International audienceMonoclonal immunoglobulin M (IgM) anti-myelin-associated glycoprotein (MAG) neuropathy is a rare disabling condition, most commonly treated with rituximab monotherapy (R), which leads to neurological improvement in only 30%-50% of patients. The combination of rituximab plus chemotherapy has been proven to improve the level of responses. We studied the outcomes of anti-MAG neuropathy patients treated either by R, or by immunochemotherapy (ICT) in our centre, focusing on the incidence of the first neurological response evaluated by the modified Rankin Scale (mRS). From 2011 to 2018, 64 patients were studied: 34 were treated with R and 30 with ICT. According to our treatment decisionmaking process, the median mRS was higher in the ICT group (mRS 2) compared to the R group (mRS 1). At 1 year, mRS improvement rates were 46% and 18% of the ICT and R groups of patients respectively, with a median time to response of 8 and 13 months (p=0.023). Adverse effects were higher in the ICT group: 62% vs 15% (p˂0.01) all grades included. One secondary acute leukaemia occurred 5 years after treatment by ICT. In conclusion, ICT may be used as a valid option for patients with rapidly progressive and/or severe anti-MAG neuropathy symptoms

Cerebrospinal fluid interleukin (IL)-10 and IL-10:IL-6 ratio as biomarkers for small B-cell lymphoproliferations with leptomeningeal dissemination

International audienceWe here report for the first time that low levels of interleukin (IL)-10 do not exclude lymphomatous meningitis (LM) in B-cell lymphoproliferative disorders (CLPD). Unexpectedly, IL-10 levels and IL-10:IL-6 ratio in CLPD differed from the levels observed in diffuse large B-cell lymphoma (DLBCL). We report the usefulness of adding the IL-10:IL-6 ratio in order to potentially reveal more aggressive lymphomas: either a transformation or an association with another “hidden” lymphoma such as primary CNS lymphoma (PCNSL)

Genetic characterization of B-cell prolymphocytic leukemia: a prognostic model involving and MYC and TP53

B-cell prolymphocytic leukemia (B-PLL) is a rare hematological disorder whose underlying oncogenic mechanisms are poorly understood. Our cytogenetic and molecular assessment of 34 patients with B-PLL revealed several disease-specific features and potential therapeutic targets. The karyotype was complex ({greater than or equal to}3 abnormalities) in 73% of the patients and highly complex (5 abnormalities) in 45%. The most frequent chromosomal aberrations were translocations involving [t()] (62%), deletion (del)17p (38%), trisomy (tri)18 (30%), del13q (29%), tri3 (24%), tri12 (24%), and del8p (23%). Twenty-six of the 34 patients (76%) exhibit aberration, resulting from mutually exclusive translocations or gains. Whole-exome sequencing revealed frequent mutations in , , , , , , , , and The majority of B-PLL used the or subgroups (89%), and displayed significantly mutated genes (79%). We identified three distinct cytogenetic risk groups: low-risk (no aberration), intermediate-risk ( aberration but no del17p), and high-risk ( aberration and del17p) (p=.0006). drug response profiling revealed that the combination of a B-cell receptor or BCL2 inhibitor with OTX015 (a bromodomain and extra-terminal motif (BET) inhibitor targeting ) was associated with significantly lower viability of B-PLL cells harboring a t(). We conclude that cytogenetic analysis is a useful diagnostic and prognostic tool in B-PLL. Targeting may be a useful treatment option in this disease

Flow cytometry minimal residual disease after allogeneic transplant for chronic lymphocytic leukemia

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Direction du Système d'Information Connexion au réseau wifi eduroam

International audienceVoir également : Choix du réseau wifi à l'université de Rennes 1 Première connexion Repérer le réseau eduroam, puis s'y connecter : Le réseau eduroam est basé sur une authentification 802.1x, au niveau réseau : l'authentification de l'utilisateur est demandé dès la connexion au réseau sans-fil

CYCLON and NPM1 Cooperate within an Oncogenic Network Predictive of R-CHOP Response in DLBCL

International audienceR-CHOP immuno-chemotherapy significantly improved clinical management of diffuse large B-cell lymphoma (DLBCL). However, 30\textendash 40% of DLBCL patients still present a refractory disease or relapse. Most of the prognostic markers identified to date fail to accurately stratify high-risk DLBCL patients. We have previously shown that the nuclear protein CYCLON is associated with DLBCL disease progression and resistance to anti-CD20 immunotherapy in preclinical models. We also recently reported that it also represents a potent predictor of refractory disease and relapse in a retrospective DLBCL cohort. However, only sparse data are available to predict the potential biological role of CYCLON and how it might exert its adverse effects on lymphoma cells. Here, we characterized the protein interaction network of CYCLON, connecting this protein to the nucleolus, RNA processing, MYC signaling and cell cycle progression. Among this network, NPM1, a nucleolar multi-functional protein frequently deregulated in cancer, emerged as another potential target related to treatment resistance in DLBCL. Immunohistochemistry evaluation of CYCLON and NPM1 revealed that their co-expression is strongly related to inferior prognosis in DLBCL. More specifically, alternative sub-cellular localizations of the proteins (extra-nucleolar CYCLON and pan-cellular NPM1) represent independent predictive factors specifically associated to R-CHOP refractory DLBCL patients, which could allow them to be orientated towards risk-adapted or novel targeted therapies