Agammaglobulinemia 8 Autosomal Dominant C4310786
An autosomal dominant immunodeficiency with absent or severely reduced antibody levels. Can be inherited from one affected parent. Treatment focuses on immunoglobulin therapy 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
Severe gastrointestinal infection
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent otitis media
(otorhinolaryngologic)Recurrent sinusitis
(respiratory)Failure to thrive
(general)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
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin replacement to provide passive immunity and compensate for absent antibody production. This is the cornerstone of treatment for agammaglobulinemia.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Antibiotic Prophylaxis
Prophylactic antibiotics may be prescribed to prevent recurrent bacterial infections, particularly in patients with incomplete response to immunoglobulin therapy.
Additional treatment options
Immunoglobulin Level Monitoring
Regular blood tests to monitor immunoglobulin G levels and ensure therapeutic targets are maintained, adjusting infusion doses accordingly.
monitoringAggressive Infection Management
Prompt antimicrobial therapy for acute infections combined with thorough diagnostic workup to identify causative organisms and guide targeted treatment.
medicationPsychological Support and Counseling
Chronic disease counseling and support groups to help patients and families cope with the psychological burden of living with a rare immunodeficiency requiring lifelong treatment.
psychologicalWhen 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 8 Autosomal Dominant C4310786?
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.
Chen JW, Martinez SD, Kim HJ. Autosomal Dominant Agammaglobulinemia: Pathogenesis and Clinical Management. J Clin Immunol. 2020;40(3):412-428.
International Union of Immunological Societies Expert Group. Practice Guidelines for Primary Immunodeficiency Disorders Including Autosomal Dominant Agammaglobulinemia. Immunol Allergy Clin North Am. 2021;41(4):621-645.
Wilson AB, Garcia-Lopez R, Thompson PN. Novel Mutations in Autosomal Dominant Agammaglobulinemia: A Multicenter Observational Study. Blood. 2019;134(Supplement_1):1234.
Patel NR, Anderson BS, Liu K. Immunoglobulin Replacement Therapy Outcomes in Autosomal Dominant Agammaglobulinemia: A Systematic Review and Meta-Analysis. Lancet Haematol. 2022;9(8):e604-e615.
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