Lymphoma and Neuropathy
Lymphoma and Neuropathy
Abstract & Commentary
By Michael Rubin, MD, FRCP(C), Professor of Clinical Neurology, Weill Cornell Medical College, New York, NY. Dr. Rubin reports that he receives grant/research support from Pfizer and is on the speaker's bureau of Athena Diagnostics.
Synopsis: In patients with lymphoma, demyelinating neuropathy is usually associated with Hodgkin's lymphoma, whereas polyradiculopathy is usually caused by high-grade B-cell lymphoma.
Source: Viala K, et al. Neuropathy in lymphoma: a relationship between the pattern of neuropathy, type of lymphoma and prognosis? J Neurol Neurosurg Psychiatry 2008;79:778-782.
Polyneuropathy may be associated with lymphoma. The best described syndrome has positive IgM anti-myelin-associated glycoprotein (MAG) activity, resulting in a syndrome of slow onset sensory gait ataxia, with progressive symmetric ascending numbness, less pronounced weakness, and minimal pain or autonomic involvement. Electrodiagnostic studies and nerve biopsy in these patients demonstrate a predominantly demyelinating neuropathy with some axonal degeneration. But what about lymphoma patients without anti-MAG antibodies? Is there a different pattern of neuropathy in these cases? Can a specific type of neuropathy be related to a specific form of lymphoma? Is their prognosis better or worse?
Among 400 patients presenting with lymphoma, with or without neuropathy, to the Hospital de la Salpetriere, Paris, France, between 1995 and 2005, 26 patients, 13 men and 13 women, had neuropathy that was unrelated to drug treatment, anti-MAG antibodies, or other etiologies associated with neuropathy. All 26 had undergone neurologic consultation with electrodiagnostic studies (EDS), comprised of nerve conduction study (NCS) and needle electromyography; based on these evaluations, they were classified according to their pattern of neuropathy. Demyelinating polyneuropathy was based on weakness or sensory symptoms in 2 or more limbs, combined with demyelinating features on EDS. Radiculopathy was based on a root distribution of sensory and motor symptoms and signs, in the absence of demyelinating abnormalities on EDS. Axonal multiple mononeuropathy required a distal axonal asymmetric motor or sensory neuropathy in a specific nerve distribution; however, axonal distal polyneuropathy required distal symmetric motor or sensory abnormalities, again in the absence of demyelination on EDS. Neurologic recovery was defined as a complete resolution of neuropathic symptoms and signs, while improvement or worsening were defined, respectively, as an increase or decrease of at least 2 grades in the Medical Research Council (MRC) scale for strength, combined with lessening or worsening of sensory symptoms and pain. Full-thickness open nerve and muscle biopsy, performed in 11, was evaluated for inflammatory vascular lesions, cellular infiltrates, and immunochemistry.
Demyelinating polyneuropathy was diagnosed in 13 patients (50%); all of these were associated with Hodgkin's lymphoma. Radiculopathy, due to lymphomatous root infiltration, was seen in 7 (27%) and was strongly associated with high grade B cell lymphoma. Axonal multiple mononeuropathy due to distal nerve infiltration or paraneoplastic microvasculitis was present in 6 (23%). Chemotherapy, prednisone, plasma exchange, and/or intravenous immunoglobulin (IVIG) resulted in neurologic improvement in approximately 70% of those with demyelinating neuropathy, 30% of those with radiculopathy, and 50% with axonal neuropathy, and hematological remission in approximately 45%, 30%, and 85%, respectively. In lymphoma-associated neuropathy, demyelinating polyneuropathy carries the best neurologic prognosis, whereas radiculopathy resulting from proximal tumor infiltration is usually due to B cell lymphoma and carries the worst prognosis.
Apart from Hodgkin's and non-Hodgkin's lymphoma, other rare lymphomas that may affect peripheral nerves include intravascular large B-cell lymphoma, also termed angiotrophic lymphoma or malignant angioendotheliomatosis. This lymphoma is confined to the blood-vessel lumen, usually presenting as a stroke, but also as mononeuropathy or cauda equina syndrome. Lymphomatoid granulomatosis, an angiocentric and angiodestructive lymphoproliferative disorder due to Epstein Barr virus, is an infection rather than a malignant lymphoma; it presents as pulmonary and brain lesions, with neurological symptoms in up to 30%, though neuropathy is rare. Chronic lymphocytic leukemia and Waldenström's macroglobulinemia cause polyneuropathy by nerve infiltration, autoimmune attack, or amyloid deposition in nerves. Osteosclerotic myeloma and Castleman's disease (angiofollicular lymph node hyperplasia), a nonmalignant lymphoproliferative syndrome, also cause neuropathy; it is often accompanied by monoclonal gammopathies, and may demonstrate multiorgan involvement that is characteristic of Crow-Fukase or POEMS (polyneuropathy, organomegaly, endocrinopathy, M spike, and skin manifestations) syndrome.In patients with lymphoma, demyelinating neuropathy is usually associated with Hodgkin's lymphoma, whereas polyradiculopathy is usually caused by high-grade B-cell lymphoma.
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