T-B- severe combined immunodeficiency

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T-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

D81.1ICD-10 names this disease exactly — shared with 8 other rare diseases

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

MONDO 0017855MONDO 17855UMLS C5679893

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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