Agammaglobulinemia 4 Autosomal Recessive C3150752
A genetic condition resulting in near-absent antibody production, leading to recurrent severe infections. Managed with lifelong immunoglobulin replacement therapy and proactive infection prevention. Caused by mutations inherited from both parents.
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 respiratory tract infections
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent sinusitis
(respiratory)Chronic diarrhea due to Giardia
(gastrointestinal)Severe eczema
(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
Immunoglobulin Replacement Therapy
Regular immunoglobulin G replacement therapy administered intravenously every 3-4 weeks or subcutaneously weekly. This replaces the deficient antibody production and provides protection against encapsulated bacteria and common pathogens.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Antibiotic Prophylaxis
Prescription of prophylactic antibiotics such as trimethoprim-sulfamethoxazole or azithromycin to prevent recurrent sinopulmonary infections that commonly occur in agammaglobulinemia patients.
Additional treatment options
Acute Infection Management
Aggressive treatment protocol for any acute bacterial infections including early cultures, targeted antibiotic therapy, and close follow-up to ensure complete resolution and prevent complications.
procedureComprehensive Immunodeficiency Monitoring
Serial monitoring of IgG trough levels, complete blood counts, liver function tests, and periodic pulmonary function testing to assess treatment efficacy and detect complications early.
monitoringNutritional Support and Immunonutrition
Balanced diet rich in immune-supportive nutrients including adequate protein, vitamins A, C, D, E, zinc, and selenium. Consider supplementation for any documented deficiencies to support overall immune function.
dietaryWhen 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 4 Autosomal Recessive C3150752?
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.
Bonilla FA, Khan DA, Ballas ZK, et al. Practice Parameter for the Diagnosis and Management of Primary Immunodeficiency. J Allergy Clin Immunol Pract. 2015.
Conley ME, Broides A, Hernandez-Trujillo V, et al. Genetic Analysis of Patients with Agammaglobulinemia. Curr Opin Allergy Clin Immunol. 2012.
Chapel H, Cunningham-Rundles C. Update on the Immunoglobulin Treatment for Agammaglobulinemia. J Clin Immunol. 2019.
Quesada MP, Marquez J, Hernandez-García M. Molecular Basis of Autosomal Recessive Agammaglobulinemia. Immunol Allergy Clin North Am. 2020.
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