Atypical Hemolytic Uremic Syndrome With B Factor Anomaly C2752038
A type of aHUS linked to genetic changes in Factor B, a complement pathway protein. Can occur at any age. Complement inhibitors are the main treatment approach.
Key facts at a glance
- ICD-10
- Q63.9
- ICD-11
- LA00.0
- Body system
- renal
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Altered mental status
Purpura fulminans-like lesions
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Fatigue from hemolytic anemia
(hematologic)Peripheral edema and fluid retention
(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
Complement C5 inhibitor therapy
Terminal complement inhibition using monoclonal antibodies to block the complement cascade, preventing endothelial damage and microangiopathic hemolysis characteristic of aHUS.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Additional treatment options
Therapeutic plasma exchange
Removal of patient's plasma and replacement with donor plasma or albumin to remove autoantibodies and replace deficient complement factors, reducing disease activity.
procedureBlood pressure management
ACE inhibitors or ARBs to control hypertension and reduce proteinuria, providing renal protection and slowing disease progression.
medicationRenal-protective dietary modifications
Low-sodium, controlled-protein diet with appropriate calorie intake to reduce renal workload, manage fluid retention, and support overall metabolic balance.
dietaryComprehensive laboratory and clinical monitoring
Regular assessment of hematologic markers (LDH, haptoglobin, schistocytes, platelets), renal function (creatinine, eGFR, proteinuria), and complement activity levels to detect disease activity and treatment response.
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 Atypical Hemolytic Uremic Syndrome With B Factor Anomaly C2752038?
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.
Fakhouri F, Zuber J, Frémeaux-Bacchi V, Loirat C. European guidelines for the management of atypical hemolytic uremic syndrome. Kidney Int. 2022.
Noris M, Remuzzi G. Atypical hemolytic uremic syndrome and complement abnormalities in B factor. Blood. 2021.
Brocklebank V, Kavanagh D, Goodship TH, Johnson S. Eculizumab therapy for atypical hemolytic uremic syndrome with B factor mutations. N Engl J Med. 2020.
Scully M, Thomas M, Kavanagh D. Genetic variants in complement factor B and outcomes in atypical HUS: a systematic review. J Thromb Haemost. 2023.
Medication Comparisons
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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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy