Soliris successfully manages pregnancy-related aHUS in woman
Case study: Early diagnosis allows for timely treatment measures
A 26-year-old woman who developed pregnancy-associated atypical hemolytic uremic syndrome (p-aHUS) successfully recovered following treatment with Soliris (eculizumab), according to a case report from China.
This case provides “a valuable reference for the timing, efficacy, and safety of complement blockade in p-aHUS [pregnancy-associated aHUS],” according to researchers. They added that the “successful use of eculizumab in this setting is especially notable given the limited data and experience with complement inhibition for p-aHUS in Asian populations.”
The case was described in “Early Identification and Eculizumab Treatment of Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Case Report,” which was published in Cureus.
Pregnancy can be a trigger for aHUS
The abnormal activation of a part of the immune system known as the complement pathway causes aHUS. This results in blood clotting in small vessels, or thrombotic microangiopathy, and can lead to symptoms such as low levels of red blood cells due to their destruction (hemolysis), low levels of platelets that aid in blood clotting, and acute kidney failure.
Although pregnancy can be a trigger for aHUS, there’s often an underlying genetic predisposition.
In this report, researchers described the case of a woman who went to the hospital at 38 weeks of pregnancy due to a 30-minute episode of low back pain. At the initial examination, her blood pressure was significantly elevated (hypertension), and an emergency ultrasound suggested placental abruption, or when the placenta separates from the inner wall of the uterus before birth.
She underwent an emergency cesarean section, delivering a baby boy. After the surgery, she reported severe headache and dizziness. Exams further showed hypertension, hemolysis, elevated liver enzymes, low platelet counts, and acute kidney injury.
HELLP syndrome initially suspected
HELLP (Hemolysis, Elevated Liver enzymes, and Low Platelets) syndrome was initially suspected and she was transferred to the intensive care unit. Despite continuous kidney replacement therapy, her creatinine levels, a measure of kidney function, continued to increase.
“The clinical presentation of p-aHUS often overlaps considerably with other pregnancy-related [thrombotic microangiopathies], particularly HELLP syndrome … posing significant diagnostic challenges,” the researchers wrote.
A negative Coombs test excluded an autoimmune condition with antibodies targeting red blood cells, and stool tests excluded infections, suggesting a diagnosis of aHUS. Subsequent tests indicated normal ADAMST13 enzyme activity, which ruled out thrombotic thrombocytopenic purpura, but no mutations in aHUS-related genes.
This case emphasizes that when pregnant women have hypertension and related complications, p-aHUS should be considered as one of the differential diagnoses.
The woman began plasma exchange, a procedure in which plasma, the liquid part of blood, is replaced to remove harmful antibodies, on the fourth day after surgery. She started on Soliris four days later at a dose of 900 mg weekly. Due to the risks of meningococcal disease, a serious bacterial infection, she received meningococcal vaccination and ceftriaxone, an antibiotic.
Her urine output increased, and creatinine levels rapidly declined. She was discharged 22 days after the cesarean section and continued to be monitored as an outpatient for kidney function while continuing on Soliris.
The treatment was discontinued after her kidney function normalized, and she remained relapse-free in the last three months of follow-up.
“This case emphasizes that when pregnant women have hypertension and related complications, p-aHUS should be considered as one of the differential diagnoses,” the researchers wrote. “Early diagnosis allows timely treatment measures, which can improve patient prognosis.”
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