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Updated: May 10, 2026
06:29
Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
Published on: January 29, 2014
A complementary component to atypical haemolytic uraemic syndrome
Vanita Gandhi1, Aine Burns, Timothy Goodship
1Department of Nephrology, Royal Free Hospital NHS Trust, London, UK. vanita.gandhi@doctors.org.uk
BMJ Case Reports
|June 20, 2013
Summary
Atypical hemolytic uremic syndrome (aHUS) in a young man was linked to a CD46 mutation. Treatment led to complete recovery and no recurrence within a year, highlighting effective aHUS management.
Area of Science:
- Nephrology
- Hematology
- Genetics
Background:
- Atypical hemolytic uremic syndrome (aHUS) is a rare, severe thrombotic microangiopathy.
- Genetic mutations in complement regulatory proteins are implicated in aHUS pathogenesis.
Observation:
- A 22-year-old male presented with clinical manifestations consistent with aHUS.
- Genetic analysis revealed a mutation in the CD46 complement regulatory protein.
Findings:
- The patient received plasma exchange, intermittent hemodialysis, and blood transfusions.
- Complete hematological and renal function recovery was observed at 6-month follow-up.
- No disease relapse occurred in the subsequent year.
Implications:
- This case underscores the importance of genetic analysis in diagnosing aHUS.
- Successful management with standard therapies suggests a favorable prognosis for CD46-associated aHUS.
- Further research into complement-targeted therapies for aHUS is warranted.
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