Development of Biclonal Gammopathy in a Patient with von Recklinghausen's Neurofibromatosis

Wille, Leif E.; Ostborg, J

doi:10.1111/j.0954-6820.1979.tb06038.x

Cited by 3 publications

(2 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In patient 111-1 a monoclonal gammopathy was observed initially. During three years' observation, a biclonal gammopathy developed (44). These observations strongly suggest a familial syndrome which, to our knowledge, has not been reported before.…”

Section: Discussionsupporting

confidence: 67%

See 1 more Smart Citation

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

Wille

Førre

Steffensen

1980

Journal of Internal Medicine

Self Cite

View full text Add to dashboard Cite

Three cases of von Recklinghausen's disease have been observed in the same family comprising four members. Two of the patients, father and son, had aorta oufflow obstruction and biclonal gammopathy (IgG(K/h) and IgA(K)/IgG(K)). In one Of these patients, no concanavalin suppressor cell activity was demonstrated, indicating that the gammopathy may be related to suppressor T-cell deficiency. Further study of the other family lineages showed that aorta stenosislmors subita occurred frequently, but genetic marker studies failed to reveal any linkage between these entities. The syndrome, which has not been reported previously, was probably restricted to the f i t kindred studied. Detailed biochemical and immundogical studies of the monoclonal components involved are in progress.

show abstract

Section: Discussionsupporting

confidence: 67%

“…In Oct. 1975, a monoclonal IgG(kappa) paraprotein was detected after agarose gel electrophoresis. Two years later he had developed a biclonal gammopathy of IgG(kappa) and IgG(1ambda) specificities, respectively (44). Biopsy of excised skin tumor material verified the presence of neurofibromas.…”

Section: Patient 111-imentioning

confidence: 97%