Factor H Deficiency C0398777
A rare immune system disorder affecting complement regulation. Patients have increased susceptibility to infections and may develop kidney problems.
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 hemolytic anemia
Thrombotic microangiopathy leading to organ failure
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Recurrent infections with encapsulated bacteria
(immunologic)Glomerulonephritis
(renal)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.
Complement inhibitor therapy
Eculizumab or ravulizumab therapy targeting terminal complement to prevent complement-mediated tissue damage characteristic of Factor H deficiency.
Plasma-derived factor replacement
Fresh frozen plasma or specialized plasma products containing functional Factor H to provide complement regulatory activity in acute or prophylactic settings.
Additional treatment options
Complement and renal function monitoring
Regular laboratory monitoring including CH50, complement levels, renal function tests, and markers of hemolysis to assess disease control and treatment response.
monitoringInfection prevention and immunization
Aggressive infection prevention through vaccinations, prophylactic antibiotics when indicated, and prompt treatment of infections given increased susceptibility.
lifestyleSupportive renal care
Nephrology coordination for renal protection including blood pressure management, proteinuria monitoring, and early intervention for renal involvement.
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 Factor H Deficiency C0398777?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy