severe combined immunodeficiency

ICD-10: D81.0MeSH: D016511ORPHA: 33355

Overview

combined T cell and B cell immunodeficiency that is caused by a defect in several genes encoding for B and T lymphocytes resulting in individuals with non-functional immune systems

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with severe combined immunodeficiency, sourced from HPO and Orphanet clinical annotations.

Hearing impairmentChronic otitis mediaSkin rashFailure to thriveWeight lossAbnormality of neutrophilsDecreased total leukocyte countAnemiaDehydrationFeverDiarrheaMalabsorptionRecurrent respiratory infectionsAbnormality of mitochondrial metabolismDecreased circulating immunoglobulin concentrationSevere combined immunodeficiencyCellular immunodeficiencyAplasia/Hypoplasia of the thymusSepsisSkin ulcerLymphopenia

Classification & Codes

ICD-10 Code

D81.0

MeSH Code

D016511

Orphanet Code

ORPHA:33355
severe combined immunodeficiency
ICD-10D81.0
MeSHD016511
OrphanetORPHA:33355
Treatments0 drug(s)
Symptoms on record21 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO