Ultomiris successfully treats pregnancy-associated aHUS in woman, 31: Report
After 'uneventful' pregnancy, condition developed shortly after baby's birth
A woman in her early 30s who developed pregnancy-associated atypical hemolytic-uremic syndrome (aHUS) after the birth of her baby saw her kidney function recover after being treated with Ultomiris (ravulizumab-cwvz), according to a case report.
Clinicians diagnosed the rare condition a week after her first symptoms — which included anemia and signs of kidney injury — after ruling out multiple other potential causes. The patient was then started on Ultomiris, which improved her kidney function and increased her platelet counts.
“Early administration of [Ultomiris] after diagnosis of [pregnancy-associated]-aHUS may contribute to favorable clinical outcomes and recovery of renal function,” the researchers wrote, noting that no further signs of the disease were seen for this patient.
The report, “Pregnancy-Associated Atypical Hemolytic Uremic Syndrome Successfully Treated with Ravulizumab: A Case Report,” was published in the journal Cureus.
No reported recurrence for patient after stopping Ultomiris treatment
aHUS belongs to a group of disorders called thrombotic microangiopathies, or TMAs, which are characterized by red blood cell destruction known as hemolysis, low platelet counts, and the formation of blood clots inside small blood vessels. These blood clots may damage internal organs, particularly the kidneys, with about half of patients developing end-stage kidney disease.
Most people with aHUS have genetic mutations in genes regulating the activity of the complement cascade, a part of the immune system, that causes it to become overactive. However, a trigger event usually is also necessary for the disease to develop.
Such trigger events can include pregnancy, certain diseases, or medications.
Here, researchers described the case of a 31-year-old woman who developed pregnancy-associated aHUS following the birth of her child, and in whom treatment with Ultomiris “resulted in a favorable clinical course.”
[Ultomiris] is an effective treatment for [pregnancy-associated] aHUS, and initiating administration early after diagnosis may contribute to favorable clinical outcomes and recovery of renal function.
The woman became pregnant after undergoing fertility treatment. The pregnancy was “uneventful,” according to the team, with the baby born full term by cesarean section. The mother was transferred to the researchers’ intensive care unit (ICU) due to uterine bleeding, which was successfully controlled by blocking an artery supplying blood to the uterus.
On the day after her admission, the patient started showing signs of anemia, or low red blood cell counts, as well as low platelet counts and acute kidney injury. Platelets are small cell fragments in the blood that form clots and stop or prevent bleeding.
These symptoms, together with elevated levels of lactate dehydrogenase, a marker of tissue damage, led to the suspicion of TMA. That TMA diagnosis was later confirmed after a blood smear revealed the presence of red blood cell fragments consistent with hemolysis.
The woman started treatment with prednisolone, plasma exchange, and hemodialysis — a treatment that takes over the kidneys’ function of filtering blood.
After excluding infections, autoimmune diseases, or other conditions that could potentially cause TMA, the doctors ultimately diagnosed the patient with pregnancy-associated aHUS.
Consequently, treatment with Ultomiris, a long-acting antibody that specifically targets the C5 complement protein to suppress the activity of the complement system, was started.
That treatment led to increases in the woman’s platelet counts, which reached normal levels seven days after she was started on the therapy; it also improved her kidney function. Hemodialysis was discontinued after six sessions, and the woman was discharged after 28 days of treatment.
As an outpatient, the woman continued receiving Ultomiris, given three times every eight weeks. After treatment was stopped, she had no disease recurrences. Genetic testing conducted later did not reveal the presence of aHUS-related mutations.
“[Ultomiris] is an effective treatment for [pregnancy-associated] aHUS, and initiating administration early after diagnosis may contribute to favorable clinical outcomes and recovery of renal function,” the researchers wrote.
However, considering the rarity of pregnancy-associated aHUS, the team noted that additional studies assessing the use of Ultomiris in such patients are necessary to confirm these findings.
About the Author
