Agammaglobulinemia 3 Autosomal Recessive C3150751
An inherited immune disorder causing severely reduced antibody production due to defective B cells. Patients experience frequent bacterial infections. Treatment involves regular immunoglobulin infusions and infection prevention measures.
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
Autoimmune cytopenia
Severe sepsis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent bacterial infections
(respiratory)Absence of tonsils/lymphoid tissue
(lymphatic)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
Intravenous or subcutaneous immunoglobulin replacement to provide passive immunity against common pathogens. This is the cornerstone of treatment for agammaglobulinemia, replacing the absent antibodies that the body cannot produce.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Antibiotic Prophylaxis
Long-term prophylactic antibiotics to prevent bacterial infections, particularly respiratory tract infections. Typically used alongside immunoglobulin therapy to provide comprehensive infection prevention.
Additional treatment options
Infection Management Protocol
Prompt evaluation and treatment of any infections with appropriate antibiotics based on culture and sensitivity results. Establish clear action plans for fever and signs of infection.
procedureImmunological and Infection Monitoring
Regular monitoring of immunoglobulin levels, infection frequency, and growth/development parameters. Periodic assessment of lung function and imaging to detect early complications.
monitoringInfection Prevention Practices
Implementation of hygiene practices to reduce exposure to infections including frequent hand washing, avoiding crowded enclosed spaces during peak respiratory seasons, and avoiding contact with obviously ill individuals.
lifestyleWhen 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 3 Autosomal Recessive C3150751?
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, Farmer DM, Smith CI. Advances in understanding autosomal recessive agammaglobulinemia. Immunol Allergy Clin North Am. 2020.
Bonilla FA, Barlan I, Chapel H, et al. Practice parameter for the diagnosis and management of primary immunodeficiencies. J Allergy Clin Immunol. 2019.
Tsuchiya S, Yoshikawa K, Hara A. Novel mutations in BTK gene causing autosomal recessive agammaglobulinemia type 3. J Clin Immunol. 2022.
Al-Herz W, Bousfiha A, Casanova JL, et al. Primary immunodeficiency diseases: a molecular and genetic approach. Clin Immunol. 2021.
Medication Comparisons
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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