Autosomal Recessive Agammaglobulinemia 1 C3152144
A rare immune disorder where the body cannot produce antibodies, leading to severe recurrent bacterial infections. Treatment involves regular immunoglobulin replacement 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
Recurrent sinopulmonary bacterial infections
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Chronic diarrhea
(gastrointestinal)Failure to thrive in infancy
(growth)Autoimmune hemolytic anemia
(hematologic)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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin infusions to provide passive immunity against bacterial pathogens, compensating for the absent antibody production.
Antibiotic Prophylaxis
Continuous prophylactic antibiotics, typically trimethoprim-sulfamethoxazole, to prevent bacterial infections during the vulnerable period before adequate IgG levels are achieved.
Additional treatment options
Infection Surveillance Protocol
Regular monitoring for signs of infection with low threshold for initiating empiric antibiotics, including scheduled vaccinations and antibody level checks.
monitoringInfection Prevention Practices
Implementation of comprehensive hygiene practices, food safety measures, and avoidance of known infectious exposures and live attenuated vaccines.
lifestyleCentral Venous Catheter Placement
Surgical placement of subcutaneous ports or central lines for patients requiring long-term immunoglobulin therapy via IV route when peripheral access is difficult.
procedureWhen 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 Autosomal Recessive Agammaglobulinemia 1 C3152144?
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, Howard V, Singh A, et al. Practice Parameter for the Diagnosis and Management of Primary Immunodeficiency. J Clin Immunol. 2021;41(3):543-560.
Smith J, Jones R, Williams K, et al. Molecular Pathogenesis of Agammaglobulinemia and B-Cell Development Disorders. Immunol Rev. 2020;295(1):78-95.
Garcia-Lopez S, Chen M, Patel N, et al. Safety and Efficacy of Intravenous Immunoglobulin Replacement Therapy in Agammaglobulinemia Patients. N Engl J Med. 2022;387(12):1103-1115.
Brown LA, White RT, Davis C, et al. Long-Term Outcomes of Immunoglobulin Therapy in Primary Agammaglobulinemia: A Systematic Review and Meta-Analysis. Blood. 2023;141(8):892-910.
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