Monoclonal Gammopathy in Hereditary Spherocytosis: A Possible Pathogenetic Relation

Abstract

Two cases of monoclonal gammopathy in patients with hereditary spherocytosis indicated a possible pathogenetic relation between these 2 disorders. Twelve adult patients with hereditary spherocytosis had significant hyper .gamma.-globulinemia in comparison to normal subjects. Retrospective analysis of previous illness in 140 patients with multiple myeloma showed a significant association between Ig[immunoglobulin]A myeloma and previous gallbladder disease. The chronic reticuloendothelial stimulation may be due to extravascular hemolysis, possibly potentiated by the inflammation associated with cholelithiasis and cholecystitis, and may foster neoplastic transformation of immunocytes in patients with hereditary spherocytosis, ultimately leading to the development of monoclonal gammopathy.