["Benign" monoclonal gammopathy and chronic lymphatic leukemia in a patient with Noonan syndrome].
METHODS
At the age of 32 a "benign" monoclonal gammopathy of lightchain kappa with Bence Jones protein is diagnosed in a man born 1934. In addition a Noonan-syndrome is found.


RESULTS
Twenty-four years later he gradually develops a chronic lymphatic leukaemia (B-CLL) which up to now does not need treatment (October 1996). The neoplastic B-cells exprime monoclonal lightchain lambda on the cellmembrane and in the cytoplasma undetectable by immunefixation in the serum. Irrespective of that the known monoclonal gammopathy exprimes IgG-kappa without an increase in the number of plasmacells in the bonemarrow.


CONCLUSIONS
There are hints that the congenital Noonan-syndrome can be associated with B-cell disorders.
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Riederer
D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology; D001536:Bence Jones Protein / Q000378:metabolism; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood; D007145:Immunoglobulin kappa-Chains / Q000097:blood; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000235:genetics* / Q000276:immunology; D008297:Male; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000235:genetics* / Q000276:immunology; D009634:Noonan Syndrome / Q000235:genetics* / Q000276:immunology; D010950:Plasma Cells / Q000276:immunology
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D001536:Bence Jones Protein