BENIGN MONOCLONAL GAMMOPATHY AND CHRONIC LYMPHATIC-LEUKEMIA FOUND IN A PATIENT WITH NOONAN-SYNDROME

History: At the age of 32 a ''benign'' monoclonal gammopathy of lightc hain kappa with Bence Jones protein is diagnosed in a man born 1934. I n addition a Noonan-syndrome is found. Course: Twenty-four years later he gradually develops a chronic lymphatic leukaemia (D-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 cy toplasma undetectable by immunefixation in the serum. irrespective of that the known monoclonal gammopathy exprimes IgG-kappa without art in crease in the number of plasmacells in the bonemarrow. Conclusion: The re are hints that the congenital Noonan-syndrome can be associated wit h B-cell disorders.