T B Severe Combined Immunodeficiency Due To Cd3delta Cd3epsilon Cd3zeta C5679578
A severe genetic disorder where infants lack functional T and B cells due to defective T cell receptor components. Without early treatment, it is life-threatening; bone marrow transplant or gene therapy may be curative.
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
Maternal T-cell engraftment
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Failure to thrive
(immunologic)Absent thymic shadow on imaging
(immunologic)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
Hematopoietic Stem Cell Transplantation
HSCT remains the primary curative option for CD3 complex-deficient SCID, restoring both T and B cell function through donor immune cell reconstitution.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
lifelong immunoglobulin replacement necessary to compensate for combined T and B cell defects characteristic of CD3-associated SCID variants.
Additional treatment options
CD3-Specific Immune Monitoring
Specialized monitoring of CD3 complex integrity and T cell receptor signaling to assess residual immune function and treatment response.
monitoringEnhanced Infection Prophylaxis
Aggressive antimicrobial prophylaxis addressing viral, bacterial, and fungal pathogens common in T cell deficiencies including PJP and CMV.
medicationNutritional Support
Optimized nutrition and micronutrient supplementation to support immune function, growth, and overall health in severely affected patients.
complementaryWhen 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 T B Severe Combined Immunodeficiency Due To Cd3delta Cd3epsilon Cd3zeta C5679578?
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.
Fischer A, Le Deist F, Hacein-Bey-Abina S. Severe combined immunodeficiency due to CD3 complex deficiency. Immunol Allergy Clin North Am. 2015.
Buckley RH, Pai SY, Routes JM. Practice guidelines for severe combined immunodeficiency. J Clin Immunol. 2020.
Markert ML, Devlin BH, McCarthy EA. CD3delta deficiency: clinical outcomes in 42 patients. Blood. 2018.
Notarangelo LD, Puck JM. T-B-NK+ SCID variants. In: Stiehm ER editor. Immunologic Disorders in Infants and Children. 6th ed. Philadelphia: Elsevier; 2019.
Medication Comparisons
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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