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Start free with EleplanT-B- severe combined immunodeficiency
ORPHA:317419Clinical group
Also called T-B- SCID
What it is
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.
Key facts
- Classified as
- Clinical group
Recorded for the broader condition
- Prevalence
- 1-9 / 100 000 (at birth, Europe)Severe combined immunodeficiency
- Age of onset
- Infancy, NeonatalSevere combined immunodeficiency
- Inheritance
- Autosomal recessive, X-linked recessiveSevere combined immunodeficiency
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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