Neurological manifestations of Waldenström macroglobulinemia.
Waldenström macroglobulinemia, a condition that most commonly occurs in lymphoplasmacytic lymphoma, typically manifests with diffuse lymphadenopathies, cytopenias, and a markedly elevated erythrocyte sedimentation rate. Peripheral neuropathy occurs in nearly half of patients with this condition, and hyperviscosity-related nervous system disorders are encountered in up to a third. Other neurological complications, such as encephalopathy or myelopathy caused by direct tumor infiltration, paraprotein deposition or autoimmune phenomena, are rare. Diagnosis of Waldenström macroglobulinemia requires identification of monoclonal IgM protein in the serum, bone marrow biopsy, and appropriate neurological testing (e.g. imaging studies of affected areas of the central neuraxis, electrophysiological studies). Treatment options, which should address both the paraprotein burden and the lymphoplasmacytic clone, include plasmapheresis and chemotherapy with alkylating agents, nucleoside analogs, and rituximab. As the disease is incurable and its course indolent, these treatments are only provided to symptomatic patients.
Baehring
Joachim M
JM
Yale University School of Medicine, Yale Brain Tumor Center, New Haven, CT, USA.
Hochberg
Ephraim P
EP
Raje
Noopur
N
Ulrickson
Matthew
M
Hochberg
Fred H
FH
D006801:Humans; D009422:Nervous System Diseases / Q000209:etiology* / Q000503:physiopathology*; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000628:therapy