Gallbladder surgery triggers aHUS in woman, 20: Case study
Patient, who was treated with Soliris, had recently given birth
A previously healthy 20-year-old woman developed atypical hemolytic uremic syndrome (aHUS) after having surgery to remove her gallbladder shortly after giving birth, according to a recent case study.
The woman was treated successfully with Soliris (eculizumab) and with no complications. The case shows “minimally invasive surgeries done within the postpartum period could be a precipitator for HUS [hemolytic uremic syndrome],” the researchers wrote. The study, “Cholecystectomy-induced thrombotic microangiopathy (TMA) in a postpartum patient successfully treated with eculizumab: a case report,” was published in the Journal of Medical Case Reports.
Thrombotic microangiopathy (TMA) encompasses a group of diseases marked by low platelet counts, red blood cell destruction (hemolysis), and organ damage due to blood clots forming in small blood vessels. A type of TMA, aHUS is caused by the abnormal activation of the complement cascade, a part of the immune system that normally helps clear microbes and damaged cells from the body.
Most aHUS cases are considered primary because they are caused by genetic mutations that disrupt the regulation of the complement cascade. Secondary aHUS occurs when the disease arises in association with another illness or triggering event, generally in the absence of clear, underlying complement defects. Pregnancy is a known event for triggering aHUS in up to 20% of cases.
A trigger for aHUS
Scientists at the University of California, Irvine Medical Center described a case of aHUS that was triggered by the removal of the gallbladder — a procedure called a cholecystectomy — in a previously healthy woman who’d recently given birth.
Two months after giving birth, the 20-year-old woman was seen by clinicians complaining of abdominal pain lasting four days and showing elevations in biomarkers of liver injury. An abdominal ultrasound showed dilated bile ducts, which are specialized channels in the liver that carry bile, a digestive fluid essential for breaking down fats, from the liver and gallbladder to the intestines. Further tests revealed a bile duct stone, which was removed.
The woman underwent a laparoscopic cholecystectomy to remove the gallbladder, but the procedure was complicated by a dramatic drop in platelet counts and levels of hemoglobin, the protein that carries oxygen in red blood cells. She also developed acute kidney injury.
The woman was transferred to the intensive care unit, where blood tests indicated anemia and hemolysis, and were consistent with kidney and liver injury. She was started on hemodialysis due to poor kidney function and low urine output.
During the first five days in the hospital, she had low blood oxygen levels, called hypoxia, and required high-flow oxygen supplementation. She also had an elevated white blood cell count accompanied by a fever. She was given antibiotics to treat a possible pneumonia and/or an abdominal infection, given her recent surgery.
Her medical team worried she might be developing TMA, so she was given the approved aHUS therapy Soliris, starting on day five and then once weekly. Her fever resolved after a few days and her white blood cell count returned to normal after a week. A genetic analysis found variants in several complement-related genes, but the woman tested negative against a complement-mediated TMA panel.
On day five, a kidney ultrasound showed signs of abnormalities and a subsequent kidney biopsy showed signs of TMA. Complement-mediated TMA was confirmed and she continued weekly treatment with Soliris.
The woman’s condition improved throughout her admission and no complications were seen while she received treatment. She was discharged on day 20, with plans to continue dialysis twice weekly, along with weekly Soliris infusions. During a follow-up, she had no complaints and her kidney function improved to the normal range. Soliris treatment was continued for a year.
“Our case emphasizes the importance for clinicians to have a high degree of clinical awareness and judgement surrounding complement-mediated TMA, aHUS and its subsets, and most importantly surgery as a precipitator regardless of [the] relative safety of the procedure, especially during the postpartum period,” the researchers wrote.
About the Author
