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Standard first-line therapy was started due to ongoing neuropathy, despite being otherwise asymptomatic and having low-risk assessment based on the international prognostic scoring system (IPSSWM 0).</p><p id="par0010" class="elsevierStylePara elsevierViewall">He presented symmetric weakness and atrophy of both extensor and flexor muscle groups, more pronounced in gastrocnemius. Osteotendinous reflexes were abolished. Sensory examination showed a severe defect of the small and large sensory fibers. Nerve conduction studies showed a demyelinating sensory and motor polyneuropathy.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Given the long evolution time and coexisting plasma cell dyscrasia with production of monoclonal IgM component, diagnosis approach was addressed to distinguish between direct neural infiltration by lymphoplasmacytic cells from amyloid neuropathy. Therefore, a muscle and nerve biopsy were performed showing a pattern of denervated muscle whereas nerves showed large deposits of amorph material (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A, arrows) consistent with amyloid deposits in the Congo-red stain. Positive Congo-red staining was observed in the endoneurium (stars) and perineurium vessels (arrows) (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Immunohistochemical stains revealed marked positivity for kappa light chains, concordant with the plasmatic M component, leading to final diagnosis of AL amyloid neuropathy.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Ethical approval</span><p id="par0030" class="elsevierStylePara elsevierViewall">Article based on the description of a real clinical case. The protocols of our center regarding the use of patient data for dissemination purposes have been followed, as well as the guidelines for the anonymization of identifying data.</p><p id="par0035" class="elsevierStylePara elsevierViewall">We have the verbal consent of the patient to present in sessions and publish the case for educational or informative purposes about her pathology. 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Image in medicine
Amyloid polyneuropathy in a patient with Waldenström macroglobulinemia
Polineuropatía amiloidea en un paciente con macroglobulinemia de Waldenström
Daniel N. Marcoa, Iban Aldecoab,c, Daniel Sanchez-Tejerinad, José C. Milisendaa,
, Raquel Aranegaa
Autor para correspondencia
a Muscle Research Unit, Internal Medicine Service, Hospital Clínic de Barcelona (HCB), Universidad de Barcelona and Center for Biomedical Research on Rare Diseases (CIBERER), Barcelona, Spain
b Department of Pathology, Biomedical Diagnostic Centre (CDB), Hospital Clínic de Barcelona, University of Barcelona, Spain
c Neurological Tissue Bank of the Biobank-IDIBAPS, Hospital Clínic de Barcelona, University of Barcelona, Spain
d Department of Neurology, Hospital Universitari Vall d’Hebrón (HVH), Barcelona, Spain