Plasma exchange therapy managed aHUS in elderly man: Report
Pancreas inflammation triggered rare disease in 72-year-old in China
Plasma exchange therapy successfully managed atypical hemolytic uremic syndrome (aHUS) triggered by pancreas inflammation in a 72-year-old man, according to a case report from China.
However, discontinuation of plasma exchange and a transition to hemodialysis — a treatment used to remove waste and excessive fluid from the blood when the kidneys are not functioning normally — led the patient to develop chronic kidney failure, per the report.
That result led the researchers to note the need for effective treatments for better outcomes in aHUS patients.
“Hemodialysis cannot replace [plasma exchange] in aHUS patients, and early, accurate, and adequate treatment is crucial for prognosis,” the researchers wrote.
The man’s case was detailed in a study titled “Atypical hemolytic uremic syndrome triggered by acute pancreatitis,” which was published as a letter to the editor in The Spanish Journal of Gastroenterology.
Plasma exchange therapy discontinued due to cost
aHUS is caused by the excessive activation of the complement cascade — a group of immune proteins that normally help fight off infections — leading to the formation of blood clots inside small blood vessels. The disease is marked by a triad of symptoms: hemolytic anemia, which occurs when red blood cells are destroyed faster than they are produced; thrombocytopenia, or low levels of the platelets that normally help the blood to clot; and acute kidney failure.
The factors behind complement activation, such as infections and pregnancy, can also act as triggers for aHUS. Pancreas inflammation, known as pancreatitis, also can serve as a trigger for complement activation and potentially for the rare condition.
Here, researchers described the case of an older man with a history of untreated gallstones in whom aHUS arose due to pancreatitis.
The man sought hospital treatment for abdominal pain that had been lasting one day. A CT scan showed signs of acute pancreatitis and gallstones.
After 12 hours of routine treatment, doctors detected the presence of hemoglobin — the protein that carries oxygen in red blood cells — in the man’s urine and feces. Further examination indicated acute kidney injury, hemolytic anemia, thrombocytopenia, and blood clotting dysfunction.
The patient was started on plasma exchange therapy, a treatment in which plasma, the liquid part of blood, is removed and replaced to eliminate disease-driving complement components.
In this case, the patient improved significantly after [plasma exchange], but insufficient treatment led to serious sequelae, [or aftereffects, in this case, kidney failure].
Lab tests indicated the man had high levels of lactate dehydrogenase, a marker of cell and tissue damage, fragmented red blood cells in blood smears, and low levels of the C3 complement protein, These findings led to a diagnosis of aHUS caused by acute pancreatitis.
After three plasma exchange therapy sessions, the man’s condition gradually improved. However, due to financial constraints, the patient discontinued plasma exchange, opting for hemodialysis instead, and eventually progressed to chronic kidney failure.
“In this case, the patient improved significantly after [plasma exchange], but insufficient treatment led to serious sequelae,” or aftereffects, in this case kidney failure, the researchers wrote.
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