Peripheral neuropathies and lymphoma without monoclonal gammopathy: a new classification

Abstract

Recent progress in immunopathological studies of peripheral nerve and lymph node fragments together with 16 personal cases and numerous clinicopathological reports have suggested a new classification of peripheral neuropathies (PN) and lymphomas. These are: (1) PN due to local infiltrations by a T-cell lymphoma: (2) acute polyradiculoneuritis due to active demyelination and associated with infiltrates of a T-cell lymphoma in the epineurium, resembling Marek's disease (which is a T-cell lymphoma); (3) B-cell lymphoma proliferation which may be restricted to or predominate in the peripheral nervous system, with a large clinicopathological heterogeneity ranging from localized forms to ascending polyradiculoneuropathies; (4) angiotropic lymphoma, which is a B-cell lymphoma and may present as an acute mononeuropathy; (5) patients with acquired immunodeficiency syndrome due to lymphomatous infiltrates in the endoneurium, of which 2 cases of PN have been reported; (6) PN associated with organomegaly, endocrinopathy, M-component and skin lesions, certain cases being associated with a plasmocytoma and sometimes Castleman's disease but without any monoclonal gammopathy: (7) classic Guillain-Barré syndrome, prone to develop in patients with extraneural lymphoma but without any lymphomatous infiltrates in the peripheral nervous system; (8) certain cases (4 out of 16 in our series) where there is no clear relationship between PN and lymphoma, and there are mainly features of axonal degeneration. Inflammatory perivascular infiltrates were sometimes present in the epineurium.

Clinical Reasoning: A 47-year-old woman with left shoulder pain after a fall

“…2 The initial description by Lhermitte and Trelles of peripheral neuropathy due to lymphomatous invasion of the peripheral nerves was published in 1934. 3 A later study described 10 cases with nervous system involvement out of 7,000 autopsies on patients with lymphoma. 4,5 Most lymphomatous invasions are difficult to diagnose.…”

“…Proposed mechanisms of this presentation seen in 7% of patients include possible lymphoma cell neurotropism, which could infiltrate from adjacent lymph nodes and spread along nerves. 1,3,7 The incidence of concomitant CNS involvement in cases of NL is seen in 10% of patients with NL. 7 Common features that may suggest NL include severe aching, burning pain (resulting in insomnia and lack of appetite), asymmetric distribution, progressive, unrelenting onset, and rapid evolution.…”

Multifocal motor neuropathy caused by a B‐cell lymphoma producing a monoclonal IgM autoantibody against peripheral nerve myelin glycolipids GM1 and GD1b

“…Some patients who have extraneural lymphoma without any apparent lymphoma cell infiltration in the peripheral nerves develop severe multifocal motor neuropathy with active demyelination (Vital et al, 1990;Vallat et al, 1995). Reported cases have included Hodgkin's lymphoma and B-cell lymphoma; patients typically are middle-aged men with no preceding infectious disease.…”

Sensory Neuropathy as Revealing Symptom of Neurolymphomatosis: Report of a Case with a 15-Year Duration

“…Involvement of the peripheral nervous system by non-Hodgkin malignant lymphomas (NHML) is rare and may be due to various mechanisms [1,2]. Lymphomatous infiltration generally results in progressive and severe neuropathies.…”

“…Familial hemiplegic migraine (FHM) is an autosomal dominant neuronal channelopathy [1,2]. Clinical onset usually occurs during adolescence [3].…”