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Investigational Device Shows Potential for Treating Immune Dysregulation Disorders
A recent case at Mount Sinai Beth Israel suggests that an investigational device may have potential benefits as a life- saving therapy for nephrology, immunology, and hematology patients with immune dysregulation disorders.
Nikolas Harbord, MD, an Assistant Professor of Medicine (Nephrology) at the Icahn School of Medicine at Mount Sinai, saw a 39-year-old male who presented with fever, dyspnea, and lymphadenopathy. An extensive malignancy and infection workup followed, including biopsies of bone marrow, lymph nodes, and liver, resulting in a diagnosis of hemophagocytic lymphohistiocytosis (HLH), an often deadly disease that is diagnosed in one out of every 800,000 people annually but may be undiagnosed in many more.
Recognizing that the patient had a relatively poor prognosis, the nephrology and intensive care team obtained institutional review board approval for compassionate use of CytoSorb® to treat the patient. Manufactured by the CytoSorbents Corporation in New Jersey, CytoSorb is an extracorporeal cytokine adsorber approved for use in Europe in situations where cytokines are elevated, such as systematic inflammatory response syndrome, sepsis, liver failure, and post-cardiac surgery. It is not currently FDA approved.
“One of our emeritus faculty is an expert in hemoperfusion and sorbent technology and a consultant for CytoSorbents,” Dr. Harbord says. “We knew based on that experience that it could help, so we received institutional permission to use the device, and the company sent it to us.”
The patient was started on a daily six-hour regimen of combined hemodialysis/hemoperfusion, which was performed by a dialysis nurse. After the patient’s blood was cleaned through dialysis, it was passed through the CytoSorb device, where biocompatible porous polymer beads removed the cytokines. Dr. Harbord says the patient’s clinical improvement was immediate and significant, with hemodynamic stabilization during the first treatment, followed by enhanced liver function and mental status.
“We were able to demonstrate marked improvement in the patient’s clinical illness by removing the cytokines, which were responsible for his hypotension and shock,” Dr. Harbord says. “The dose of pressors,
the day-to-day bloodwork—all of it improved, and we saw a 56 percent reduction in cytokines over the course of therapy.”
Dr. Harbord says that CytoSorb is nonspecific, removing many elevated cytokines (both pro- and anti-inflammatory), but he adds that the risks are not significant and that the risk of not intervening may be greater. Given the results achieved in this investigational use, he says, cytokine hemadsorption has potential as a treatment for HLH, a disease with a mortality rate in excess of 50 percent.
“I think we demonstrated real proof-of-concept benefit for patients through investigational use,” Dr. Harbord says. “I would not hesitate to use it again, and I would hope it gives other nephrologists the confidence to adopt it based on our experience at Mount Sinai.”
