Clinical and immunological findings in four infants with Omenn's syndrome: a form of severe combined immunodeficiency with phenotypically normal T cells, elevated …
L Businco, A Di Fazio, MG Ziruolo, AL Boner… - Clinical Immunology and …, 1987 - Elsevier
We report four cases of Omenn's syndrome (OS), an autosomal recessive disease
characterized by early erythrodermia, protracted diarrhea, severe infections,
lymphadenopathy, hepatosplenomegaly, failure to thrive, and leukocytosis with marked
eosinophilia. The immunological investigations revealed B lymphopenia with increased
levels of serum IgE and marked depression of T-cell activation, not restored by the addition
of exogenous interleukin 2 (IL-2). IL-2 and interferon-γ (IFN-γ) production in vitro were very …
characterized by early erythrodermia, protracted diarrhea, severe infections,
lymphadenopathy, hepatosplenomegaly, failure to thrive, and leukocytosis with marked
eosinophilia. The immunological investigations revealed B lymphopenia with increased
levels of serum IgE and marked depression of T-cell activation, not restored by the addition
of exogenous interleukin 2 (IL-2). IL-2 and interferon-γ (IFN-γ) production in vitro were very …
