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18-11-2023 | Rituximab | Original Communication

Clinical, biological, electrophysiological and therapeutic profile of patients with anti-MAG neuropathy according to MYD88^L265P and CXCR4 mutations and underlying haemopathy

Authors: Alexandre Guérémy, José Boucraut, John Boudjarane, Aude-Marie Grapperon, Etienne Fortanier, Laure Farnault, Jean Gabert, Frédéric Vely, Romaric Lacroix, Ludivine Kouton, Shahram Attarian, Emilien Delmont

Published in: Journal of Neurology | Issue 3/2024

Abstract

Introduction

Anti-MAG neuropathies are associated with an IgM monoclonal gammopathy of undetermined significance (MGUS) or with a malignant haemopathy. Our objective was to determine whether the presence of a haemopathy or somatic mutations of MYD88 and CXCR4 genes influences disease presentation and response to rituximab (RTX).

Methods

We included 79 patients (mean age 74 years, disease duration 9.68 years) who had a bone marrow aspiration with morphologic and immunophenotypic analysis. MYD88^L265P and CXCR4 mutations were analysed in peripheral B cells.

Information collected included: inflammatory neuropathy cause and treatment sensory sum score (ISS), MRC testing, overall neuropathy limitation scale (ONLS), Rash-built Overall Disability Score (RODS), ataxia score, anti-MAG titres, peak IgM dosage, neurofilament light chain levels, motor and sensory amplitudes, motor unit index (MUNIX) and motor unit size index (MUSIX) sum scores. Efficacy of RTX was evaluated at 12 months in 26 patients.

Results

Malignant haematological disorders were discovered in 17 patients (22%): 13 Waldenstrom macroglobulinemia, 3 marginal zone lymphoma and one mantle cell lymphoma. MYD88^L265P mutation was detected in 29/60 (48%) patients and CXCR4 in 1 single patient. Disease severity, biological and electrophysiological data and response to RTX were comparable in patients with MGUS/lymphoma and patients with/without MYD88^L265P mutation. ISS was lower and MUSIX higher in patients improved by RTX.

Conclusions

MYD88^L265P mutation and underlying haemopathies are not predictive of a more severe disease. However, in cases of resistant and progressive neuropathy, they provide an opportunity to prescribe newly available drugs such as Bruton tyrosine kinase inhibitors.

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Title: Clinical, biological, electrophysiological and therapeutic profile of patients with anti-MAG neuropathy according to MYD88L265P and CXCR4 mutations and underlying haemopathy
Authors: Alexandre Guérémy
José Boucraut
John Boudjarane
Aude-Marie Grapperon
Etienne Fortanier
Laure Farnault
Jean Gabert
Frédéric Vely
Romaric Lacroix
Ludivine Kouton
Shahram Attarian
Emilien Delmont
Publication date: 18-11-2023
Publisher: Springer Berlin Heidelberg
Keywords: Rituximab
Rituximab
Lymphoma
Mantle Cell Lymphoma
Published in: Journal of Neurology / Issue 3/2024
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-023-12068-4

2024 ASCO Annual Meeting

Springer Medicine

Clinical, biological, electrophysiological and therapeutic profile of patients with anti-MAG neuropathy according to MYD88^L265P and CXCR4 mutations and underlying haemopathy

Abstract

Introduction

Methods

Results

Conclusions

2024 ASCO Annual Meeting

Springer Medicine

Abstract

Introduction

Methods

Results

Conclusions

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