Monoclonal Hypergammaglobulinemia of theγM Type in a Nine-year-old Girl with Ataxia-Telangiectasia

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Abstract
A disease pattern in a 9-year-old girl with ataxiatelangiectasia which resembled Aleutian disease of mink of the myeloma-like type is described. During the last three years of life the patient developed an electrophoretic homogeneous γM peak which probably represented a γM monoclonal gammopathy. Autopsy disclosed diffuse plasmacytosis of kidney, liver, bone marrow, and lungs, and vascular lesions of the kidneys, spleen, pancreas, and stomach wall. Prior to development of monoclonal gammopathy the patient had a herpes infection. The interrelationship between the thymic hypoplasia, the known high risk for malignancy in these patients, and their susceptibility to viral infections in the pathogenesis of the patent's disease is discussed.

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