Waldenström's macroglobulinemia in monozygotic twins

This paper reports a unique familial occurrence of Waldenström's macroglobulinemia (WM) in monozygotic twins. The determination of twin monozygosity has been performed by electrophoretic and immunological typing of genetic systems (erythrocyte blood groups, leucocyte antigens and serum protein polymorphism). The two monoclonal IgM differ one from the other by their light chain type and their idiotypic determinants. Although a genetic predisposition to WM exists in these twins, the gene recombination leading to idiotypic specificity and light chain assortment occurs independently of the monoclonal malignant involvement.