Severe cold agglutinin disease and cryoglobulinemia secondary to a monoclonal anti-Pr-2 IgM lambda cryoagglutinin

Objective To present a case of cold agglutinin disease/cryoglobulinemia secondary to a monoclonal anti-Pr-2 IgM lambda antibody, and review the literature on th e occurrence of this antibody in cold-induced disease and the clinical dise ase associated with it. Methods Cryoantibody characteristics were evaluated by cold precipitation. The anti gen specificity of the monoclonal IgM lambda antibody was evaluated using t echniques of selective red blood cell absorption. Results In our patient, we were able to identify an antibody with both cryoglobulin emic and cold agglutinin (cryoagglutinin) properties. This antibody was fou nd to be monoclonal IgM lambda with specificity to the Pr-2 antigen on red blood cells. Conclusions Monoclonal IgM lambda anti-Pr is a rarely found cold agglutinin antibody. I n this report we describe the clinical course of a patient who had this ant ibody, which not only agglutinated red cells in the cold but also had cryog lobulin properties. The clinical illness of this man was characterized by s evere acrocyanosis and digital necrosis with eventual organ necrosis and de ath. We also review the literature on cold induced disease due to monoclona l anti-Pr IgM lambda antibody. Our patient was found to be unique among the reports reviewed. Our case is the first to report both cold agglutinin and cryoglobulinemic properties with the evaluation of the thermal amplitudes of these activities of the antibody. Also, unlike the lymphoproliferative m alignancy observed in the cold agglutinin-associated disease in the other r eports, our patient's disease was associated with a monoclonal B-cell expan sion on the spectrum between benign monoclonal gammopathy and a law grade l ymphoproliferative disorder.