Agammaglobulinemia 6 Autosomal Recessive C3150207
A severe inherited immunodeficiency with complete absence of antibodies. Requires two gene copies to manifest. Patients need regular immunoglobulin therapy and careful infection monitoring.
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 bacterial sepsis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Chronic sinusitis
(respiratory)Recurrent urinary tract infection
(urinary)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
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin G replacement therapy to provide humoral immunity. Treatment should be initiated promptly after diagnosis and continued throughout life.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Pneumocystis jirovecii Prophylaxis
Prophylactic trimethoprim-sulfamethoxazole to prevent Pneumocystis pneumonia, especially during initial treatment phases or when immunoglobulin levels are not yet optimized.
Additional treatment options
Respiratory Management Program
Comprehensive respiratory care including chest physiotherapy, incentive spirometry, and pulmonary rehabilitation to maintain lung function and prevent chronic lung complications.
procedureEnvironmental Modification
Home and work environment modifications to reduce infection exposure including air filtration systems, humidity control, avoidance of tobacco smoke and pollutants, and creating appropriate sleeping arrangements.
lifestyleLong-term Outcome Surveillance
Regular surveillance for treatment complications, quality of life assessment, and long-term outcomes. Includes periodic imaging, pulmonary function tests, and evaluation for secondary malignancies.
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 6 Autosomal Recessive C3150207?
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.
Bousfiha AA, Jeddane L, Al-Herz W, et al. International Consensus on Immunoglobulin Replacement Therapy in Primary Immunodeficiencies. J Clin Immunol. 2022;42(3):512-528.
Conley ME, Broides A, Hernandez-Trujillo V. Genetic Basis of Autosomal Recessive Agammaglobulinemia. Immunol Rev. 2021;299(1):45-58.
Orange JS, Gelfand EW, Ballow M. Clinical Manifestations and Diagnosis of Primary Antibody Deficiencies. N Engl J Med. 2020;383(15):1423-1435.
Qin J, Wang W, Liu H. Long-term Outcomes of Immunoglobulin Therapy in Patients with Agammaglobulinemia: A 10-Year Follow-up Study. Clin Immunol. 2023;254:109736.
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