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T-B- severe combined immunodeficiency

rare

T-B- severe combined immunodeficiency (SCID) is a group of rare monogenic primary immunodeficiency disorders characterized by a lack of functional peripheral T and B lymphocytes, resulting in recurrent early-onset severe respiratory viral, bacterial or fungal infections, diarrhea and failure to thrive. Hypersensitivity to ionizing radiation is a characteristic feature of some of its sub-types.

Identifiers
MONDO:0017855 Orphanet:317419
Part of
Severe combined immunodeficiency
Also known as
T-B- SCID

Subtypes

All diseases Rare only
Disease Subtypes
Reticular dysgenesis 1
Cernunnos-XLF deficiency
DNA ligase IV deficiency
Omenn syndrome
Combined immunodeficiency due to partial RAG1 deficiency
Combined immunodeficiency with skin granulomas
Immunodeficiency 73b with defective neutrophil chemotaxis and lymphopenia
Immunodeficiency 73c with defective neutrophil chemotaxis and hypogammaglobulinemia
Neutrophil immunodeficiency syndrome
Reticular dysgenesis-like severe combined immunodeficiency
Severe combined immunodeficiency due to DCLRE1C deficiency
Severe combined immunodeficiency due to DNA-PKcs deficiency
Severe combined immunodeficiency due to LCK deficiency
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-positive
Short-limb skeletal dysplasia with severe combined immunodeficiency

Rare disease

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