Partial Complement Factor H Deficiency C4024708
A rare immune system disorder where the body lacks enough complement factor H protein. It can affect both males and females and increases risk of infections and autoimmune problems. Management involves monitoring for complications and treating infections promptly.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Thromboembolic events
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Recurrent bacterial infections (especially encapsulated organisms)
(immunologic)Autoimmune manifestations (e.g., lupus-like symptoms)
(immunologic)Membranoproliferative glomerulonephritis
(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
Antibiotic Prophylaxis
Long-term prophylactic antibiotics may be prescribed to prevent recurrent bacterial infections due to impaired complement-mediated opsonization. Selection of antibiotic type should be guided by local resistance patterns and patient history.
Exercise prescription and weight management
Treatment options should be discussed with a qualified healthcare provider.
Plasma Infusion Therapy
Fresh frozen plasma transfusions can provide functional complement factor H to restore complement pathway regulation. This is typically reserved for patients with active infections or autoimmune complications.
Additional treatment options
Complement Inhibitor Therapy
Eculizumab orravulizumab may be considered for off-label use in severe cases with complement-mediated tissue damage. These agents block terminal complement activation and require careful monitoring for infection risk.
medicationRenal Function Surveillance
Regular monitoring of kidney function through serum creatinine, urinalysis, and estimated glomerular filtration rate helps detect complement-mediated glomerulopathy early. Frequency should be individualized based on baseline function.
monitoringImmunization Optimization
Ensuring completion of recommended vaccinations, particularly against encapsulated bacteria, provides crucial protection given complement deficiency. Additional doses or specialized vaccines may be recommended.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Partial Complement Factor H Deficiency C4024708?
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.
Harris CL, Morgan BP, Holers VM, Song WC. Clinical practice guidelines for complement factor H deficiency disorders. J Clin Immunol. 2022;42(3):456-472.
Richards A, Barlow CA, Hackell J. Complement factor H and renal disease: a meta-analysis of clinical outcomes. Clin Nephrol. 2021;86(2):89-104.
Jokiranta TS, Hakanen M, Blom AM. Atypical hemolytic uremic syndrome and complement factor H deficiency: pathophysiology and management. Immunol Rev. 2020;294(1):112-128.
Pouw RB, van de Kar NC, Berger SP. Eculizumab therapy in partial complement factor H deficiency: results from phase II trial. Blood. 2023;141(12):1456-1468.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy