A Familial Syndrome with von Recklinghausen's Neurofibromatosis, Gammopathy and Aorta Outflow Obstruction

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Abstract
Three cases of [human] von Recklinghausen''s disease were observed in the same family comprising 4 members. Two of the patients, father and son, had aorta outflow obstruction and biclonal gammopathy (Ig[immunoglobulin]G (.kappa./.lambda.) and IgA (.kappa./IgG) (.kappa.)). In one of these patients, no concanavalin suppressor cell activity was demonstrated, indicating that the gammopathy may be related to suppressor T[thymus-derived]-cell deficiency. Further study of the other family lineages showed that aorta stenosis/mors subita occurred frequently; genetic marker studies failed to reveal any linkage between these entities. The syndrome, which has not been reported previously, was probably restricted to the 1st kindred studied.