Combined Immunodeficiency X Linked C6022467
A genetic condition primarily affecting males that impairs immune function, making patients vulnerable to recurrent infections. Treatment may include bone marrow transplant, infection prevention, and immune support.
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
Recurrent severe bacterial, viral, and fungal infections
Opportunistic infection such as Pneumocystis jirovecii pneumonia
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Failure to thrive in infancy
(immunologic)Chronic diarrhea with malabsorption
(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
Hematopoietic Stem Cell Transplantation
HSCT replaces the defective immune system with healthy donor stem cells. This is the primary curative approach for X-linked combined immunodeficiency, ideally performed before severe infections occur.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
Regular intravenous or subcutaneous immunoglobulin infusions provide passive immunity against common pathogens. This addresses the antibody deficiency component of the disease.
Additional treatment options
Antimicrobial Prophylaxis
Combination antibiotic and antiviral prophylaxis prevents opportunistic infections common in combined immunodeficiency. Trimethoprim-sulfamethoxazole is commonly used for PCP prevention.
medicationInfection Prevention Measures
Strict hand hygiene, avoiding crowds, and ensuring clean water and food reduces exposure to pathogens. Protective isolation measures are recommended during high-risk periods.
lifestyleRegular Immunological Monitoring
Serial measurement of lymphocyte subsets, immunoglobulin levels, and vaccine responses guides treatment adjustments and detects complications early.
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 Combined Immunodeficiency X Linked C6022467?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Combined Immunodeficiency X Linked C6022467. 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