Peripheral nervous system involvement in lymphomas

Abstract

The peripheral nervous system may be involved at any stage in the course of lymphoproliferative diseases. The different underlying mechanisms include neurotoxicity secondary to chemotherapy, direct nerve infiltration (neurolymphomatosis), infections, immune‐mediated, paraneoplastic or metabolic processes and nutritional deficiencies. Accordingly, the clinical features are heterogeneous and depend on the localization of the damage (ganglia, roots, plexi, and peripheral nerves) and on the involved structures (myelin, axon, and cell body). Some clinical findings, such a focal or diffuse involvement, symmetric or asymmetric pattern, presence of pain may point to the correct diagnosis. Besides a thorough medical history and neurological examination, neurophysiological studies, cerebrospinal fluid analysis, nerve biopsy (in selected patients with suspected lymphomatous infiltration) and neuroimaging techniques (magnetic resonance neurography and nerve ultrasound) may be crucial for a proper diagnostic workup.

Primary Neurolymphomatosis in the Cavernous Sinus and the Brainstem. A Case Report and Review of Literature

“…Almost all cases occur in the later stages of systemic lymphoma which secondarily affects the peripheral nerves. Primary neurolymphomatosis (PNL), i.e., without systemic lymphoma, is far less common and requires careful [5]). The majority of these patients present with cervical or lumbosacral neuropathies [6,7] [16,17].…”

Neurolymphomatosis, a rare manifestation of peripheral nerve involvement in lymphomas: Suggestive features and diagnostic challenges

“…Radiation‐induced damage was considered in the differential diagnosis, because it may present with radicular or plexus distribution, often asymmetric at onset, with predominant motor involvement and less common sensory manifestations. Despite its rarity and its possible occurrence even decades from the time of treatment, 1 it should always be considered in patients with previous radiation therapy. Consistently, our patient had initial asymmetric symptoms that presented 9 years after radiation therapy performed for DLBCL.…”

“…Unremarkable laboratory findings ruled out the possibility of an infectious radiculo‐plexopathy. Vincristine (included in the CHOP chemotherapy protocol performed to treat DLBCL) is commonly associated with peripheral sensory‐motor neuropathy occurring during treatment 1 . However, neurophysiology ruled out a possible underlying peripheral neuropathy (no abnormalities also at distal nerves, including the dorsal sural).…”

“…Neurolymphomatosis, the histologically documented infiltration of the peripheral nervous system from neoplastic lymphoid cells, represents an uncommon and often overlooked manifestation of lymphomas. Despite its rarity, neurolymphomatosis may be the first manifestation of the underlying hematological disease, often a B‐cell non‐Hogdkin lymphoma (NHL) 1 . An early diagnosis is crucial for starting proper treatment.…”

The Bruton tyrosine kinase inhibitor ibrutinib improves anti-MAG antibody polyneuropathy

“…Anti-myelin-associated glycoprotein (MAG) antibody neuropathy is a chronic sensorimotor demyelinating polyneuropathy associated with immunoglobulin M (IgM) monoclonal gammopathy, manifestation of either a monoclonal gammopathy of undetermined significance (MGUS) or of a non-Hodgkin lymphoma, such as Waldenström macroglobulinemia (WM). 1,2 Anti-MAG antibodies are pathogenic, 3 and rituximab, a chimeric anti-CD20 monoclonal antibody, is currently the standard treatment. However, rituximab provides benefit in less than 50% of patients, 4 and repeated cycles are often necessary with progressive loss of benefit.…”