Atypical Hemolytic Uremic Syndrome With Mcp Cd46 Anomaly C2752040
A genetic form of aHUS involving mutations in the MCP/CD46 complement regulator. Can affect both children and adults. Some patients may improve without treatment. Complement inhibitors are used when needed.
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
Microangiopathic hemolytic anemia
Hypertension
Confusion
Changes in urination or swelling
(renal)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hematuria
(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
Monoclonal antibodies targeting complement C5 to prevent complement-mediated endothelial damage and thrombotic microangiopathy. This therapy addresses the underlying pathophysiology of MCP/CD46 deficiency in aHUS.
Renal disease management
Active treatment with regular monitoring by a healthcare provider is recommended.
Plasma Exchange
Therapeutic plasma exchange removes autoantibodies and replaces deficient complement regulatory proteins. Used as adjunctive therapy or bridge treatment while awaiting complement inhibitor effect.
Additional treatment options
Laboratory and Renal Monitoring
Regular monitoring of hematologic parameters including LDH, haptoglobin, schistocytes, creatinine, and urine protein to detect disease recurrence and assess treatment response.
monitoringAntihypertensive Management
Blood pressure control with agents such as ACE inhibitors or ARBs to manage hypertension secondary to renal involvement and reduce cardiovascular risk in this prothrombotic condition.
medicationRenal-Protective Diet
Dietary modifications including sodium restriction, controlled protein intake, and management of potassium and phosphorus levels to support kidney function and reduce cardiovascular burden.
dietaryWhen 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 Mcp Cd46 Anomaly C2752040?
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.
Goodship TH, Cook HT, Fakhouri F, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int. 2017.
Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011.
Campistol JM, Arias M, Ariceta G, et al. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia. 2015.
Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009.
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.
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