Absence of lymphocyte ecto-5'-nucleotidase in infants with
reticuloendotheliosis and eosinophilia (Omenn's syndrome)
EW Gelfand, D McCurdy, CP Rao and A Cohen
Lymphocytes from three infants with reticuloendotheliosis and eosinophilia
( Omenn 's syndrome) and immunodeficiency were assayed for 5'-nucleotidase
activity. B and T lymphocytes from all three patients were totally
deficient in ecto-5'-nucleotidase activity, but had normal levels of
cytoplasmic 5'-nucleotidase. In contrast, cultured lymphocytes expressed
normal ectoplasmic and cytoplasmic activities, suggesting that the
lymphocyte-restricted enzyme deficiency was not likely a primary genetic
defect. The deficiency of lymphocyte ecto-5'- nucleotidase was not
associated with any abnormality of deoxynucleoside metabolism. The absence
of lymphocyte ecto-5'-nucleotidase may be a characteristic feature of this
syndrome and may help to distinguish this disease from others with similar
manifestations.
Volume 63,
Issue 6,
pp. 1475-1480,
06/01/1984
Copyright © 1984 by The American Society of Hematology
