The term paraproteinemic neuropathy describes a heterogeneous set of neuropathies characterized by the presence\nof homogeneous immunoglobulin in the serum. An abnormal clonal proliferation of B-lymphocytes or plasma cells,\nwhich may or may not occur in the context of a hematologic malignancy, produces the immunoglobulins in excess.\nIf malignancy is identified, treatment should be targeted to the neoplasm. Most cases, however, occur as monoclonal\ngammopathy of undetermined significance. Few prospective, randomized, placebo-controlled trials are available to\ninform the management of paraproteinemic neuropathies. Clinical experience combined with data from smaller,\nuncontrolled studies provide a basis for recommendations, which depend on the specific clinical setting in which\nthe paraprotein occurs. In this review, we provide a clinically practical approach to diagnosis and management of\nsuch patients.
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