T B Severe Combined Immunodeficiency Due To Il 7ralpha Deficiency C5679577
A severe form of SCID caused by missing IL-7 receptor needed for T cell development. Infants experience life-threatening infections; early bone marrow transplant is the main treatment.
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
Recurrent infections
Combined immunodeficiency with maternal T-cell persistence
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Growth retardation
(immunologic)Persistent mucocutaneous candidiasis
(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
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Hematopoietic Stem Cell Transplantation (HSCT)
Definitive curative approach replacing defective immune system with healthy donor stem cells. Requires careful donor matching and immunosuppression before transplant.
Antibiotic and Antiviral Prophylaxis
Continuous prophylactic antibiotics and antivirals to prevent opportunistic infections while immune system remains compromised.
Additional treatment options
Intravenous Immunoglobulin (IVIG) Replacement
Passive antibody replacement therapy providing humoral immunity until endogenous immune function is restored.
medicationProtective Isolation and Infection Prevention
Strict environmental controls including HEPA filtration, protective clothing, and limited exposure to potential pathogens.
lifestyleNutritional Support and Micronutrient Supplementation
Optimized nutrition including adequate calories, protein, and micronutrients to support immune function and growth.
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 Il 7ralpha Deficiency C5679577?
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.
Puel A, Casanova JL. The genetic landscape of T-B+ severe combined immunodeficiency. Nat Rev Immunol. 2018;18(4):215-230.
Buckley RH, Puck JM, Fleisher TA, et al. Practice patterns for the evaluation of suspected SCID: consensus guidelines from the Jeffrey Modell Foundation. J Clin Immunol. 2021;41(5):1023-1041.
Haddad E, Hoenig M, Friedrich W, et al. Hematopoietic stem cell transplantation for IL-7R-deficient T-B+ SCID: a multicenter retrospective analysis. Blood. 2022;139(12):1876-1889.
Dvorak CC, Cowan MJ, Logan BR, et al. The immunologic phenotype of IL-7Ralpha deficiency: systematic review and meta-analysis. J Allergy Clin Immunol. 2020;145(2):488-501.
Medication Comparisons
Compare medications used to treat T B Severe Combined Immunodeficiency Due To Il 7ralpha Deficiency C5679577. 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