Immunodeficiency 27a C4011949
A rare inherited immune disorder that impairs the body's ability to fight infections. Often diagnosed in early childhood. Management involves preventive antibiotics, immune therapies, and treating infections promptly.
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.
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent infections
(immunologic)Mycobacterial infections
(immunologic)Impaired T cell function
(immunologic)Granulomatous disease
(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
Early Hematopoietic Stem Cell Transplantation
Timely HSCT recommended given the severe phenotype. Early intervention before chronic organ damage from recurrent infections improves survival and long-term immune reconstitution.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Aggressive Immunoglobulin Replacement
High-dose IVIG therapy with higher target trough levels than typical primary immunodeficiencies. Subcutaneous route preferred for steady-state levels and reduced infusion reactions.
Additional treatment options
Broad-Spectrum Infection Prophylaxis
Comprehensive antimicrobial prophylaxis covering bacterial, viral, fungal, and atypical pathogens. May include combination regimens such as TMP-SMX, azithromycin, acyclovir, and fluconazole.
medicationStress Reduction and Immune Support
Complementary approaches including mindfulness, adequate sleep optimization, and stress management to minimize infection-triggering immunosuppression. May include supplements after provider approval.
complementaryMulti-System Surveillance Protocol
Intensive monitoring including respiratory function, nutritional status, developmental milestones, and organ-specific imaging. Includes regular vaccination response testing to document residual immunity.
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 Immunodeficiency 27a C4011949?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy