Agammaglobulinemia 5 Autosomal Dominant C3150753
A rare primary immunodeficiency with severely low or absent antibodies due to impaired B cell development. Both males and females can be affected. Treatment involves lifelong immunoglobulin replacement and infection prevention.
Key facts at a glance
- ICD-10
- R69
- ICD-11
- MA00.0
- 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 pneumonia
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent otitis media
(otorhinolaryngologic)Persistent gastrointestinal infection
(gastrointestinal)Severe skin abscess
(integumentary)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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
Immunoglobulin replacement therapy remains the primary treatment regardless of inheritance pattern. Dosing is individualized based on body weight and IgG trough levels to maintain adequate protection.
Targeted Antibiotic Therapy
Aggressive antibiotic treatment at first sign of infection with broad-spectrum coverage initially, then culture-directed therapy. Consider prophylactic antibiotics during high-risk periods.
Additional treatment options
Vaccination Guidance and Avoidance Protocol
Administration of inactivated vaccines per standard schedules; strict avoidance of all live attenuated vaccines including MMR, varicella, and oral polio. Family members should also avoid oral polio vaccine.
procedureProbiotic Supplementation
Probiotic supplementation to support gut-associated lymphoid tissue and maintain healthy microbiome, which plays a role in immune function. Choose strains appropriate for immunodeficiency patients.
complementaryDisease Complication Surveillance
Regular monitoring for complications including bronchiectasis, chronic lung disease, autoimmune manifestations, and lymphoproliferative disorders that can occur despite immunoglobulin therapy.
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 Agammaglobulinemia 5 Autosomal Dominant C3150753?
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.
Conley ME, Rohrer J, Minegishi Y. X-linked and autosomal recessive agammaglobulinemia: a spectrum of immune defects. Curr Opin Immunol. 2022.
Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2022.
Chapel H, Cunningham-Rundles C. Molecular characterization of autosomal dominant agammaglobulinemia in clinical cohorts. Clin Immunol. 2021.
Elashi AA, Al-Herz W, Segal F, et al. Immunoglobulin replacement therapy outcomes in primary agammaglobulinemia: a systematic review and meta-analysis. J Clin Immunol. 2023.
Medication Comparisons
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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