T-B+ severe combined immunodeficiency due to CD3delta/CD3epsilon/CD3zeta
ORPHA: 1691602 Treatments Available
Overview
human disease
Available Treatments (2)
| Drug | Form | Status | Countries | Lead Time |
|---|---|---|---|---|
| Immunoglobulin Cold Chain | IV infusion 10% solution (100mg/mL), subcutaneous injection 20% solution | FDA Approved | 10 | 10d |
| Hematopoietic stem cell transplantation Orphan Cold Chain | Intravenous infusion of stem cells | Standard of care | 10 | 90d |
Clinical Presentation
Signs and symptoms associated with T-B+ severe combined immunodeficiency due to CD3delta/CD3epsilon/CD3zeta, sourced from HPO and Orphanet clinical annotations.
Recurrent infectionsDecreased total lymphocyte countFeverDecreased circulating total IgADecreased circulating IgG concentrationFailure to thrive secondary to recurrent infectionsDecreased mitogen-induced T-cell proliferationDecreased total T cell countOtitis mediaErythrodermaHepatosplenomegalyIncreased total eosinophil countDiarrheaAnorexiaPneumoniaRecurrent abscess formationProtracted diarrheaRecurrent herpesRecurrent candida infectionsRecurrent pneumoniaChronic oral candidiasisIncreased circulating immunoglobulin concentrationHepatitis
Classification & Codes
Orphanet Code
ORPHA:169160T-B+ severe combined immunodeficiency due to CD3delta/CD3epsilon/CD3zeta
| Orphanet | ORPHA:169160 |
| Treatments | 2 drug(s) |
| Symptoms on record | 23 signs |
| Status | published |
Treatment Summary
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO