/severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, NK cell-negative, due to adenosine deaminase deficiency

severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, NK cell-negative, due to adenosine deaminase deficiency

Rare Disease

MONDO:0007064

Also Known As

ADAADA deficiencyADA-SCIDSCID due to ADA deficiencySCID due to ADA deficiency, early-onsetSCID due to adenosine deaminase deficiencyadenosine deaminase deficiencyadenosine deaminase deficiency, partial, Autosomal recessive, Somatic mosaicismadenosine deaminase deficient severe combined immunodeficiencysevere combined immunodeficiency due to ADA deficiency, Autosomal recessive, Somatic mosaicismsevere combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, NK cell-negative, due to adenosine deaminase deficiencySCID due to ADA deficiency, delayed onsetSCID due to ADA deficiency, late-onsetadenosine deaminase deficiency, partialpartial ADA deficiencysevere combined immunodeficiency due to ADA deficiencysevere combined immunodeficiency due to adenosine deaminase deficiency

Definition

A form of SCID characterized by profound lymphopenia and very low immunoglobulin levels of all isotypes resulting in severe and recurrent opportunistic infections.