Low-Titer Cold-Hemagglutinin Disease

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Abstract
We studied two patients with a low-titer cold-hemagglutinin disease syndrome to investigate the mechanism of hemolysis and the therapeutic response to corticosteroids. The antierythrocyte antibody was of the IgM class, had a high thermal amplitude and had enough activity at 37°C to account for the hemolysis. The capacity of peripheral blood monocytes to increase the osmotic fragility of C3-coated erythrocytes suggests that macrophage interaction with C3-coated erythrocytes explains the observed in vivo spherocytosis. Both patients responded to high-dose corticosteroids. The data suggest that the steroid effect is probably due to alteration of macrophage complement-receptor function. These studies demonstrate the importance of antibody activity at body temperature in producing hemolysis, particularly in this variant of cold-hemagglutinin disease. The response to steroids suggests the efficacy of corticosteroid therapy in alleviating hemolysis due to macrophage recognition of erythrocytes coated with IgM and C3. (N Engl J Med 296:1490–1494, 1977)