Soliris treats aHUS in man likely triggered by kidney transplant
This first use of therapy in S. Korea followed attempts with plasma exchange
For the first time in South Korea, a patient who developed atypical hemolytic uremic syndrome (aHUS) after undergoing a kidney transplant was successfully treated with Soliris (eculizumab).
“We report the first successful case of aHUS treatment in a kidney transplant recipient with early use of [Soliris] in South Korea,” the researchers wrote.
The case was reported in “The first successful eculizumab rescue therapy of a kidney transplant recipient with atypical hemolytic uremic syndrome in South Korea: a case report,” published in the Korean Journal of Transplantation.
Early use of Soliris appears ‘important for successful treatment’
aHUS is caused by the abnormal activation of part of the immune system called the complement cascade, which leads to inflammation and blood clotting in small blood vessels, especially those in the kidneys.
Patients often have underlying genetic mutations that predispose them to aHUS. Nevertheless, a trigger event usually is needed for the disease to manifest. Organ transplant is an intensively stressful experience for the body and can act as such a trigger.
Scientists in Seoul reported the case of a 32-year-old man with end-stage kidney disease caused by an inflammatory condition, called immunoglobulin A nephropathy, who underwent a kidney transplant. The kidney he received was donated by his 60-year-old mother.
On the third day after the transplant, laboratory tests revealed low levels of hemoglobin, the oxygen-carrying protein in red blood cells, and low platelet counts. These were accompanied by high levels of the cell damage marker lactate dehydrogenase and evidence of red blood cell damage.
Collectively, lab findings were consistent with thrombotic microangiopathy — the type of blood vessel damage caused by aHUS and related conditions.
The man initially was treated with steroids and plasma exchange, but these did not resolve his symptoms. Further diagnostic tests were run, including genetic screening for aHUS-related mutations.
Eighteen days after the transplant, his clinical picture was strongly suggestive of aHUS, so treatment with Soliris was initiated. Laboratory parameters gradually improved, and after three doses of Soliris the man was well enough to be discharged from the hospital.
In cases where treatments like plasma exchange are ineffective, “early [Soliris] treatment appears important for the successful treatment of aHUS,” the scientists noted.
The man received regular treatment with Soliris over a total of 27 weeks, or about six months. Treatment then was stopped, and his condition remained stable for several months.
He had a brief episode of disease recurrence about eight months later, which resolved with a short course of plasma exchange and intravenous immunoglobulin (IVIg). The transplanted kidney has continued to function normally.
“The patient discontinued [Soliris] successfully, maintaining graft function during long-term follow-up,” the scientists wrote.
Genetic testing results came back a few days after treatment with Soliris started, and no aHUS-related mutations were identified. There were, however, some mutations in blood clotting-related genes, but the clinical significance of these genetic variations was unclear.
Soliris is sold by Alexion, which was not involved in this report.