Medizinische Klinik (Munich, Germany : 1983) · 1998
["Benign" monoclonal gammopathy and chronic lymphatic leukemia in a patient with Noonan syndrome].
Клинический случайАннотация на английскомСтатьяНазвание и аннотация: Перевод в очереди
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Riederer J
Аннотация
HISTORY: 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.
COURSE: 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.
CONCLUSION: There are hints that the congenital Noonan-syndrome can be associated with B-cell disorders.
COURSE: 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.
CONCLUSION: There are hints that the congenital Noonan-syndrome can be associated with B-cell disorders.