Soliris helps manage aHUS in patient with neurological symptoms
Case study documents treatment in woman, 26
Soliris (eculizumab) helped control atypical hemolytic uremic syndrome (aHUS) in a patient whose symptoms included seizures and signs of neurological damage, according to a case report.
The researchers said the case points to “the importance of timely diagnosis and treatment of aHUS as well as the potential value of [Soliris] in improving patient outcomes.”
The case was described in the study, “Treatment of atypical hemolytic uremic syndrome with eculizumab in a patient presenting with neuropsychiatric prodrome: a case report,” published in Frontiers in Immunology.
aHUS is a rare disease in which part of the immune system, called the complement cascade, becomes overactive, leading to a triad of aHUS symptoms that include hemolytic anemia (low red blood cell counts due to excessive red blood cell destruction), thrombocytopenia (low platelet counts), and kidney injury. Damage to the nervous system is another possible manifestation of the disease.
Brain abnormalities, kidney dysfunction
Researchers in China reported the case of a 26-year-old woman who started to develop seizures following an upper respiratory tract infection.
Upon admission to the hospital, an MRI scan showed abnormalities in the patient’s brain, and biochemistry tests of the cerebrospinal fluid surrounding the brain and spinal cord revealed increased white blood cell counts. This was consistent with encephalitis (brain inflammation), but the cause wasn’t clear.
A few days after she was admitted to the hospital, the patient’s condition worsened, culminating in a deep coma.
Further tests revealed that she had hemolytic anemia and thrombocytopenia, as well as signs of kidney dysfunction. The combination of anemia and thrombocytopenia with kidney and nervous system issues led doctors to suspect the patient may have some kind of thrombotic microangiopathy (TMA), a category of diseases that encompasses aHUS and other disorders marked by inflammation and the formation of clots in small blood vessels, leading to organ damage.
Working off this suspicion, clinicians performed plasma exchange, a blood cleansing procedure in which plasma (the liquid portion of blood) is removed and replaced, and hemofiltration, a type of treatment used to remove excess fluid and waste products from the blood when the kidneys are no longer able to do so. This led to a slight improvement in the patient’s status. Additional testing ruled out other types of TMA, and the patient was ultimately diagnosed with aHUS.
“Owing to the variable and nonspecific symptoms of aHUS, no definitive diagnostic criteria exist, and clinical diagnosis relies on excluding similar diseases,” the researchers wrote.
Following the diagnosis, treatment with Soliris was initiated. Soliris is a widely approved aHUS treatment that works to block the activation of the complement cascade. The therapy is sold by Alexion AstraZeneca Rare Disease, which was not involved with the study.
Soon after starting treatment with Soliris, the patient’s condition improved markedly. Anemia and thrombocytopenia eased, seizures ceased, and brain MRI scans showed signs of improvement. The patient continues to require dialysis to support kidney function.
“aHUS should be considered in patients presenting with neurological symptoms accompanied by acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia,” the researchers wrote. “Once diagnosed, early [Soliris] administration is crucial for improved disease control and kidney function protection.”
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