Severe autoimmune hemolytic anemia with cold agglutinin and sclerodermic features--favorable response to danazol.

A rare case of severe primary autoimmune hemolytic anemia with cold agglutinin and extensive cutaneous sclerodermic changes is reported. This association has not been previously documented in the literature. The anemia was refractory to steroids but responded to danazol treatment. Danazol may be an effective therapy in some cases of autoimmune hemolytic anemia with cold agglutinin.