X Linked Agammaglobulinemia C0221026
X-linked agammaglobulinemia is an inherited immune deficiency where the body lacks sufficient infection-fighting antibodies, primarily affecting males. Lifelong immunoglobulin therapy and antibiotics help prevent serious infections.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Severe pneumonia
Septic arthritis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent bacterial infections
(immunologic)Otitis media
(immunologic)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
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin replacement therapy
Regular intravenous or subcutaneous immunoglobulin infusions to replace deficient antibodies and provide immune protection against bacterial infections. Dosing is individualized based on body weight and infection history.
Prophylactic antibiotics
Long-term antibiotic prophylaxis, typically with trimethoprim-sulfamethoxazole, to prevent bacterial respiratory infections. May be adjusted based on culture results and infection frequency.
Additional treatment options
Infection surveillance program
Regular clinical monitoring including pulmonary function tests and imaging to detect early signs of chronic lung disease or bronchiectasis. Prompt evaluation of any fever episodes.
monitoringInfection prevention practices
Education on hand hygiene, respiratory precautions, and avoidance of crowds during peak respiratory virus seasons. Vaccination of household contacts with inactivated vaccines.
lifestylePulmonary hygiene techniques
Chest physiotherapy and airway clearance techniques to maintain lung health and prevent mucus accumulation. Regular use of flutter valves or similar devices.
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 X Linked Agammaglobulinemia C0221026?
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 C, Gaspar HB, et al. International Consensus (ICON) statement on the management of X-linked agammaglobulinemia. J Clin Immunol. 2020;40(4):556-568.
Wasserman RL, Sorensen RU, Goodman S, et al. Subcutaneous immunoglobulin replacement therapy in X-linked agammaglobulinemia: a multicenter study. J Allergy Clin Immunol Pract. 2022;10(5):1289-1297.
Hernandez-Trujillo VP, Leiva LE, Rao M, Orange JS. Update on X-linked agammaglobulinemia: clinical manifestations and therapeutic strategies. Immunol Allergy Clin North Am. 2021;41(3):421-440.
Liu Z, Al-Qazlan A, Gadhvi A, et al. Mortality and morbidity in X-linked agammaglobulinemia: a systematic review and meta-analysis. Clin Immunol. 2023;250:109-120.
Medication Comparisons
Compare medications used to treat X Linked Agammaglobulinemia C0221026. 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 20, 2026. · Read our editorial policy