Immunodeficiency Due To A Classical Component Pathway Complement Deficiency C0398750
A complement system disorder where early complement proteins fail to work properly, increasing infection risk. Can affect anyone with the genetic defect. Treatment involves infection prevention and monitoring.
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
Autoimmune manifestations resembling systemic lupus erythematosus
Recurrent meningitis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent sinopulmonary infections
(immunologic)Malar rash
(dermatologic)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
Antibiotic Prophylaxis
Long-term antibiotic prophylaxis, typically with trimethoprim-sulfamethoxazole or azithromycin, to prevent recurrent bacterial infections common in complement deficiency.
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunoglobulin Replacement Therapy
Intravenous or subcutaneous immunoglobulin therapy provides passive immunity against encapsulated organisms, compensating for the inability to opsonize bacteria effectively.
Additional treatment options
Vaccination Program
Comprehensive vaccination including pneumococcal, meningococcal, and Haemophilus influenzae type b vaccines to provide active immunity against encapsulated bacteria.
procedureInfection Surveillance Protocol
Regular monitoring with periodic laboratory assessment including complete blood counts, immunoglobulin levels, and infection markers to detect complications early.
monitoringInfection Prevention Practices
Implementation of hygiene measures including hand washing, avoiding crowds during outbreaks, and prompt medical attention for fever or infection signs.
lifestyleWhen 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 Immunodeficiency Due To A Classical Component Pathway Complement Deficiency C0398750?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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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