Peripheral neuropathy in cold agglutinin disease

Cold agglutinin disease (CAD) characteristically presents as anemia and cold-induced rash. We report a man with purpura, anemia, cold agglutinins, and a sensory-autonomic polyneuropathy. Treatment with corticosteroids and plasma exchange resulted in resolution of the anemia and polyneuropathy. We propose that CAD may be associated with a reversible sensory-autonomic neuropathy in the absence of cryoglobulinemia. Although unsupported by pathologic findings, pathogenetic hypotheses for the neuropathy in CAD are similar to those proposed for cryoglobulinemic neuropathy.