An 18-year-old woman in Korea underwent a kidney transplant after developing end-stage kidney disease, for which she had been treated with dialysis. Doctors were unsure of the cause. While the kidney transplant was meant to provide relief to the woman, she experienced surprising and unexpected effects following the surgery. Dr. Patricia Inácio, PhD writes in aHUS News that the woman began coughing up blood within two days of surgery. She began urinating less, had unusually low blood pressure and a fast heartbeat, and developed a fever.

When the woman pursued medical help, doctors determined that her lungs were inflamed and bleeding. More so, the woman had low hemoglobin levels and platelet counts. A case report published in Nephron explains that the woman continued to experience worsening symptoms: hematuria (blood in the urine), tonic-clonic seizures, low urination, and recurrent lung hemorrhages. She required multiple treatments and interventions until, after 70 days, doctors chose to remove the transplanted kidney.

It was at this point that the woman underwent genetic testing to confirm what the doctors suspected: atypical hemolytic uremic syndrome (aHUS). The diagnosis was confirmed after the woman was found to have a C3 mutation. After treating the woman with Soliris, her symptoms resolved, and she was eventually allowed to go home after a secondary transplant surgery.

The researchers note that aHUS should be considered if patients present with lung bleeding and/or kidney injuries following kidney transplantation.

About Atypical Hemolytic Uremic Syndrome (aHUS)

Atypical hemolytic uremic syndrome is a rare genetic disease that impacts kidney function. This condition results from genetic predisposition that is sparked by an environmental trigger. In aHUS, tiny blood clots form in blood vessels, preventing blood from flowing to organs. This causes the kidneys to become damaged and lose function. It is 10x less common than typical hemolytic uremic syndrome. aHUS is more common in females and is often triggered by pregnancy. Symptoms associated with aHUS may include:

• Lethargy and fatigue
• Nausea and/or vomiting
• Thrombocytopenia (low platelet counts)
• Hematuria
• High blood pressure
• Diarrhea
• Irritability
• Hemolytic anemia (red blood cell destruction that causes low red blood cell counts)
• Abdominal pain
• Swelling of the lower extremities
• Stroke
• Kidney failure

If you have atypical hemolytic uremic syndrome, there are numerous treatment options available. Doctors may choose to treat aHUS with blood or platelet transfusions, drugs that expand blood vessels, dialysis, blood pressure medication, plasma therapy, or eculizumab. Since atypical hemolytic uremic syndrome affects each person differently, make sure to speak with your doctor about the best line of treatment for you.