Neurolymphomatosis: involvement of peripheral nervous system revealing hematologic malignancy, a report of nine cases.

Publisher: Wiley Country of Publication: United States NLM ID: 9704532 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1529-8027 (Electronic) Linking ISSN: 10859489 NLM ISO Abbreviation: J Peripher Nerv Syst Subsets: MEDLINE

Publication: <2010->: Hoboken, NJ Wiley
Original Publication: New York, NY : Woodland Publications, c1996-

Neurolymphomatosis*/diagnosis ; Neurolymphomatosis*/pathology ; Peripheral Nervous System Diseases* ; Hematologic Neoplasms*/complications ; Hematologic Neoplasms*/diagnosis; Humans ; Peripheral Nervous System/pathology ; Positron-Emission Tomography

Background and Aim: Neurolymphomatosis is defined as an infiltration of the peripheral nervous system (PNS) by malignant lymphoma cells. It is a rare entity and diagnosis is complicated especially when PNS involvement is the initial and leading symptom. To improve knowledge of the disorder and shorten the time to diagnosis, we report a series of nine patients without a history of hematologic malignancy, who were diagnosed with neurolymphomatosis after evaluation and workup of peripheral neuropathy.
Methods: The patients were included from the Department of Clinical Neurophysiology at Pitié Salpêtrière and Nancy Hospitals over a period of 15 years. Diagnosis of neurolymphomatosis was confirmed by histopathologic examination for each patient. We characterized their clinical, electrophysiological, biological, imaging, and histopathologic features.
Results: The neuropathy was characterized by pain (78%), proximal involvement (44%) or of all four limbs (67%), asymmetrical or with multifocal distribution (78%), abundant fibrillation (78%), a tendency to worsen rapidly, and significant associated weight loss (67%). Neurolymphomatosis was diagnosed principally on nerve biopsy (89%) identifying infiltration of lymphoid cells, atypical cells (78%), a monoclonal population (78%), and supported by fluorodeoxyglucose-positron emission tomography, spine or plexus MRI, cerebrospinal fluid analysis, and blood lymphocyte immunophenotyping. Six patients had systemic disease and three impairment limited to the PNS. In the latter case, progression could be unpredictable and may be diffuse and explosive, sometimes occurring years after a seemingly indolent course.
Interpretation: This study provides better knowledge and understanding of neurolymphomatosis when neuropathy is the initial presentation.
(© 2023 Peripheral Nerve Society.)

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Keywords: axonal neuropathy; nerve biopsy; neurolymphomatosis; non-Hodgkin lymphoma; painful neuropathy

Date Created: 20230302 Date Completed: 20230608 Latest Revision: 20230620

20250114

10.1111/jns.12541

36861226