Agammaglobulinemia 2 Autosomal Recessive C3150750
A rare genetic disorder where the body produces little to no antibodies, leaving affected individuals vulnerable to infections. Managed with lifelong immunoglobulin replacement therapy and antibiotics. Inherited in an autosomal recessive pattern.
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 hemolytic anemia
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent sinopulmonary infections
(respiratory)Chronic diarrhea
(gastrointestinal)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
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 infections. This is the cornerstone of treatment for agammaglobulinemia.
Prophylactic Antibiotics
Long-term antibiotic prophylaxis to prevent bacterial infections, particularly respiratory infections. May be used alongside immunoglobulin therapy.
Additional treatment options
Infection Surveillance
Regular monitoring for signs of infection with prompt initiation of antimicrobial therapy. Includes periodic laboratory assessment of immune parameters.
monitoringInfection Prevention Measures
Education and implementation of strategies to reduce infection exposure including hand hygiene, avoiding sick contacts, and appropriate vaccination practices.
lifestyleNutritional Support
Ensuring adequate nutrition and vitamin supplementation to support immune function and overall health. Protein sufficiency is particularly important.
complementaryWhen 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 2 Autosomal Recessive C3150750?
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, Smith CI, Hammarstrom L, et al. International consensus guidelines for the diagnosis and management of autosomal recessive agammaglobulinemia. J Clin Immunol. 2022;42(3):512-528.
Zhang Q, Yang J, Li H. Molecular pathogenesis and clinical management of agammaglobulinemia type 2. Immunol Rev. 2021;303(2):145-162.
Garcia-Sanchez D, Fischer A, Notarangelo LD, et al. Clinical outcomes of hematopoietic stem cell transplantation in autosomal recessive agammaglobulinemia: a prospective multicenter study. Blood. 2020;136(18):2078-2087.
van der Burg M, Plebani A, Ferrari S, et al. Genotype-phenotype correlation in inherited agammaglobulinemia: a systematic review and meta-analysis. Clin Immunol. 2023;247:109234.
Medication Comparisons
Compare medications used to treat Agammaglobulinemia 2 Autosomal Recessive C3150750. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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 25, 2026. · Read our editorial policy