Monoclonal gammopathy of the immunoglobulin A class in a two-year-old girl with ataxia telangiectasia.
Ataxia telangiectasia is an autosomal recessive neurologic disorder which is frequently associated with a deficiency of IgA immunoglobulin. We report an unusual case of monoclonal gammopathy of the IgA kappa type in a 2-year-old female patient newly diagnosed with ataxia telangiectasia. Quantitative analysis of the patient's immunoglobulins revealed a marked elevation in the IgA fraction with a value of 672 mg/dL (normal 14-123 mg/dL). The IgG and IgM fractions were normal. Serum protein electrophoresis showed a band of restricted mobility present in the gamma region, which was identified as a monoclonal IgA kappa immunoglobulin on immunofixation electrophoresis. This is the first case report of a patient with ataxia telangiectasia associated with an IgA monoclonal gammopathy.
McDonald
P S
PS
Department of Pathology, George Washington University Hospital, Washington, DC 20037, USA.
Cora-Bramble
D
D
De Palma
L
L
D001260:Ataxia Telangiectasia / Q000150:complications*; D001797:Blood Protein Electrophoresis; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007070:Immunoglobulin A; D010265:Paraproteinemias / Q000150:complications*
D007070:Immunoglobulin A