Use of drug Soliris successfully treats woman’s pregnancy-linked aHUS: Case
Report highlights 'critical role' of managing condition in pregnant patients
Use of the drug Soliris (eculizumab) effectively treated atypical hemolytic uremic syndrome (aHUS) in a pregnant woman in her 30s who developed the rare disease after having placental abruption, which occurs when the placenta separates from the inner wall of the uterus before birth.
Her case was described in a new report by a team of researchers in the U.S. and Pakistan, who noted that the use of Soliris — a drug approved in the U.S. in 2011 — led to “a remarkable improvement in the maternal condition without adverse fetal outcomes.”
“This case report highlights the critical role of timely recognition and intervention in the management of aHUS during pregnancy,” the researchers wrote.
“Clinicians must consider aHUS in the differential diagnosis of pregnant patients presenting with [microangiopathic hemolytic anemia] and renal [kidney] impairment,” the team wrote.
The case report, “Successful Management of Atypical Hemolytic-Uremic Syndrome in Pregnancy Using Eculizumab: A Case Review,” was published in the journal Cureus.
Woman, 36, found to have aHUS after baby’s C-section delivery
aHUS is a form of thrombotic microangiopathy. This is a group of diseases characterized by red blood cell destruction, called hemolytic anemia, low platelet counts, and the formation of blood clots inside small blood vessels that can cause organ damage, most commonly in the kidneys.
Most people with aHUS have genetic mutations in genes that cause the complement cascade, a part of the immune system, to become overly active. However, a trigger event usually is also necessary for the disease to develop. Such trigger events can include pregnancy, certain diseases, or the use of some medications.
Now, a team of researchers described the case of a 36-year-old woman who developed aHUS during her pregnancy, and in whom treatment with the drug Soliris markedly improved her clinical condition.
The report detailed a previous history of recurrent miscarriages, elective abortion, and antiphospholipid syndrome — a condition that makes the blood more likely to clot. The patient’s clinical history also included chronic obstructive pulmonary disease (COPD), vitamin B12 deficiency, and pulmonary embolism, or a blood clot that blocks or restricts blood flow in the lungs.
After experiencing premature rupture of membranes — the medical term for when the water breaks too early in the course of a pregnancy — caused by the position of the fetus inside the womb, the woman went to an emergency department for a C-section.
Following surgery, she developed complications, including pelvic hematoma, or an accumulation of blood in the lower area of the abdomen, and kidney swelling. This led to severe anemia, or low red blood cell numbers.
The woman needed to receive 15 blood transfusions, several transfusions of platelets, and fresh frozen plasma. She also required a nephrostomy tube to help drain her urine, and the placement of an inferior vena cava filter to stop blood clots from going to her lungs.
Further lab tests revealed the presence of red blood cell fragments known as schistocytes in a blood smear, consistent with red blood cell destruction, low platelet counts, and kidney damage.
Her kidney function, low platelet levels, and hemolytic anemia continued to worsen, raising doctors’ concerns about the formation of blood clots. One week after seeking treatment, a kidney biopsy revealed inflammation in the kidneys’ filtering units, and blood clots inside small blood vessels.
These findings were consistent with the presence of aHUS. Without improvements, she underwent plasma replacement therapy, which stabilized her platelet counts.
Researchers ‘advocate’ for use of drug Soliris in pregnancy treatment
The woman then was transferred to a tertiary center for specialized close monitoring, where she started treatment with Soliris.
After the second dose of the Soliris drug, her clinical condition significantly improved, with a consistent increase in platelet counts. She also had a decrease in the levels of creatinine, a marker of kidney dysfunction, following treatment.
The woman was discharged home with instructions to complete the course of treatment with Soliris. The plan was for her to receive 900 mg of the therapy weekly for four weeks and then 1,200 mg on week five, followed by 1,200 mg every two weeks until treatment was decided to be halted based on her blood test results.
This case adds to the growing body of evidence supporting the safety and efficacy of [Soliris] in pregnancy, which may pave the way for more robust, future prospective studies.
She also was vaccinated against meningococcal bacteria, due to the risk of meningitis associated with treatment.
“This case adds to the growing body of evidence supporting the safety and efficacy of [Soliris] in pregnancy, which may pave the way for more robust, future prospective studies,” the researchers wrote.
“We advocate for including [Soliris] in treatment guidelines for aHUS in pregnancy, supported by further research to strengthen and validate our findings,” they concluded.
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