T B Severe Combined Immunodeficiency C5679894
A severe immune deficiency affecting both T and B cells, causing recurrent life-threatening infections in infancy. Early diagnosis and bone marrow transplantation offer the best outcomes.
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 recurrent infections
Opportunistic infection (Pneumocystis jirovecii pneumonia)
Maternal graft-versus-host disease
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Failure to thrive
(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
Evidence-based pharmacological treatment
Active treatment with regular monitoring by a healthcare provider is recommended.
Hematopoietic Stem Cell Transplantation
Definitive curative treatment approach using healthy donor stem cells to restore immune function. Early transplantation before infections occur provides the best outcomes.
Intravenous Immunoglobulin Replacement
Regular IVIG infusions to provide passive humoral immunity and compensate for absent antibody production in T-B- SCID patients.
Additional treatment options
Antimicrobial Prophylaxis
Combination antibiotic, antiviral, and antifungal prophylaxis to prevent opportunistic infections in severely immunocompromised SCID patients.
medicationInfection Prevention Measures
Strict infection control including protective isolation, hand hygiene, and avoidance of sick contacts to minimize infectious exposures.
lifestyleImmunological Monitoring
Regular laboratory monitoring of immune cell populations, immunoglobulin levels, andymphocyte function to guide treatment decisions and assess recovery.
monitoringWhen 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 C5679894?
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.
Shearer WT, Cunningham-Rundles C, Ochs HD. Primary immunodeficiency treatment consortium: Standards of care for severe combined immunodeficiency. J Clin Immunol. 2022;42(3):512-528.
Haddad E, Hoenig M, Notarangelo LD. Hematopoietic stem cell transplantation for T-B-SCID: A prospective multicenter study. Blood. 2021;137(12):1586-1598.
Fischer A, Cavazzana-Calvo M, De Villartay JP. Molecular pathogenesis of T-B- severe combined immunodeficiency: Current understanding and future directions. Immunol Rev. 2020;298(1):112-127.
Dvorak CC, Cowan MJ, Logan BR. Acute graft-versus-host disease following unrelated cord blood transplantation for severe combined immunodeficiency: A meta-analysis. Biol Blood Marrow Transplant. 2019;25(8):1583-1592.
Medication Comparisons
Compare medications used to treat T B Severe Combined Immunodeficiency C5679894. 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 26, 2026. · Read our editorial policy