Why has plasma exchange failed in TRACK syndrome? Lessons from a new variant of the atypical hemolytic uremic syndrome.

J Clin Apher

Department of Pediatrics, Division of Pediatric Intensive Care Unit, Sancaktepe Sehit Prof. Dr. Ilhan Varank Training and Research Hospital, University of Health Science, Istanbul, Turkey.

Published: October 2023

AI Article Synopsis

  • Atypical hemolytic uremic syndrome (aHUS) is a rare but serious illness that affects the blood and organs, making it very dangerous.
  • A 4-year-old girl with aHUS had problems with her heart because of a gene mutation, and treatments usually used, like plasma exchange, didn’t help her.
  • It's important to know that plasma exchange might not work for everyone with aHUS, especially if their illness is caused by genetic issues.

Article Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening form of thrombotic microangiopathy, associated with high mortality and morbidity. Most cases present with hemolytic anemia, thrombocytopenia, and renal insufficiency. However, it can have unusual multiple end-organ injuries including extrarenal organ and system involvements such as neurologic, cardiac, gastrointestinal, and respiratory systems. We describe a 4-year-old girl who developed aHUS due to the TSEN2 mutation and had cardiac involvement. She did not benefit from plasma exchange, as stated in previous cases. It should be kept in mind that therapeutic plasma exchange may not be beneficial in some cases of aHUS, especially due to genetic mutations.

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Source
http://dx.doi.org/10.1002/jca.22070DOI Listing

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