Abstract
Persistently increased IgA is a rare clinical observation, occurring in liver cirrhosis (all immunoglobulins increased) and myeloma (IgA is monoclonal). We have observed 4 patients with IgA greater than 300 IU/ml (normal range 25-200 IU/ml) with no evidence of liver dysfunction or myeloma; IgG and IgM values were normal, as were in vitro responses to PHA and ConA. Surprisingly, each patient showed variable aneuploidy in PHA-stimulated blood cell cultures. Fifty metaphases were counted and at least five karyotypes prepared for each patient.
Three patients displayed aberrations such as translocations, quadriradial formation, chromosome additions and deletions, affecting no specific group; one patient showed 18p- in all cells, provoking interest because of reports of IgA decrease in association with 18p-. Chromosome aberrations and profound immunodeficiency occur in Bloom's syndrome, Fanconi's anemia, ataxia telangiectasia and xeroderma pigmentosum. The high IgA values reported here may therefore reflect a basic stem cell defect, manifested as lymphoid cell aneuploidy.
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Segal, D., Pabst, H. & McCoy, E. ELEVATED IgA LEVELS ACCOMPANIED BY CHROMOSOME ABERRATIONS. Pediatr Res 8, 395 (1974). https://doi.org/10.1203/00006450-197404000-00331
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DOI: https://doi.org/10.1203/00006450-197404000-00331