Oligo-/monoclonal gammopathy and hypergammaglobulinemia in ataxia-telangiectasia. A study of 90 patients.
We investigated the presence of hypergammaglobulinemia and oligo-/monoclonal gammopathy in 90 patients (from 80 families) with ataxia-telangiectasia ranging in age from 2 to 29 years. Of the 90 patients, 38.8% displayed hypergammaglobulinemia. An isolated increase in IgM was the most common finding (23.3%) followed by a simultaneous increase in IgM and IgG (8.8%), an isolated increase in IgA (3.3%), an elevated level of IgG (2.2%) and a concomitant increase in IgM and IgA (1.1%), respectively. Seven of the patients (8.1%) had oligo-/monoclonal gammopathy. The gammopathies included all major immunoglobulin isotypes. Chemotherapeutic intervention in 2 cases precipitated the emergence of new clones within a matter of weeks. Further investigation of oligo-/monoclonal gammopathies in these patients may lead to a clearer understanding of the clinical course and provide further insight into the underlying mechanisms of B-cell abnormalities in ataxia-telangiectasia.
Sadighi Akha
A A
AA
Division of Immunology, Johns Hopkins University, School of Medicine, Baltimore, Maryland, USA.
Humphrey
R L
RL
Winkelstein
J A
JA
Loeb
D M
DM
Lederman
H M
HM
D000293:Adolescent; D001260:Ataxia Telangiectasia / Q000150:complications*; D001798:Blood Proteins / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D004200:Diseases in Twins; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000209:etiology*; D007132:Immunoglobulin Isotypes / Q000097:blood; D008297:Male; D009369:Neoplasms / Q000150:complications; D010265:Paraproteinemias / Q000097:blood / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D016176:T-Lymphocyte Subsets / Q000473:pathology
D001798:Blood Proteins; D007132:Immunoglobulin Isotypes