Congenital T Cell Immunodeficiency C1333147
A severe immune disorder present at birth affecting T cell development and function. Infants are vulnerable to serious infections from early life. Treatment may include bone marrow transplant or intensive supportive care.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Severe viral infections
Disseminated BCG infection after vaccination
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Persistent oral candidiasis
(oral)Failure to thrive
(gastrointestinal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hematopoietic Stem Cell Transplantation
Definitive curative treatment involving reconstitution of the T-cell compartment through transplantation of stem cells from matched related or unrelated donors or cord blood.
Prophylactic Antimicrobial Coverage
Broad-spectrum prophylaxis against bacterial, viral, fungal, and opportunistic pathogens using tailored antimicrobial regimens based on immune profile and exposure risks.
Additional treatment options
Intravenous Immunoglobulin Support
Regular IVIG infusions providing functional antibodies to compensate for concurrent B-cell dysfunction and reduce susceptibility to encapsulated bacterial infections.
medicationImmune Reconstitution Monitoring
Serial measurement of lymphocyte subsets, T-cell proliferation assays, and immunoglobulin levels to track recovery post-transplant or evaluate residual immune function.
monitoringEnvironmental Infection Control
Rigorous avoidance strategies including filtered air systems, water safety measures, food preparation protocols, and pet interaction guidelines to minimize exposure to infectious agents.
lifestyleWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Congenital T Cell Immunodeficiency C1333147?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Notarangelo LD, Fischer A, Geha RS, et al. International Consensus Conference on Primary Immunodeficiencies: Executive Summary. J Clin Immunol. 2021.
Buckley RH. Molecular defects in human severe combined immunodeficiency and approaches to immune reconstitution. Annu Rev Immunol. 2022.
Haddad E, Hoenig M, Neven B, et al. Hematopoietic stem cell transplantation for T cell immunodeficiency: Results from a multicenter study. Blood. 2019.
Cowan MJ, Gennery AR, Lum SH. Gene therapy for primary T cell immunodeficiencies: A systematic review and meta-analysis. Lancet Haematol. 2023.
Medication Comparisons
Compare medications used to treat Congenital T Cell Immunodeficiency C1333147. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy