Gamma heavy chain disease and rheumatoid arthritis

Abstract

G AMMA HEAVY CHAIN disease (7HCD). is characterized by the presence of incomplete immunoglobulin heavy chains in serum and/or urine’; although often described as a lymphoproliferative disorder, it has a wide variety of clinical and pathologic features.*-5 More than 60 cases have been reported”’ since Franklin et al first described this condition in 1964.3 A substantial number of cases yHCD are associated with autoimmune disorders such as rheumatoid arthritis (RA), systemic lupus erythematosus, Sjogren’s syndrome, and myasthenia gravis.2 yHCD varies in severity, whether associated with RA or not, from a benign condition, to one leading to progressive deterioration and death.4 We report a patient with severe RA preceding by 4 years the development of THCD, followed by recurrent sepsis, progressive deterioration, and death. At autopsy a non-Hodgkin’s lymphoma was found. The literature on the association between yHCD and RA is reviewed.