Hemophagocytic Lymphohistiocytosis as a complication of immune checkpoint inhibitor therapy for sarcoma

Abstract

Summary: Immune checkpoint inhibitors are an emerging therapy for metastatic sarcomas that have failed conventional therapies such as methotrexate, doxorubicin, and cisplatin. Much is still not known about the toxicities that these treatments would cause in sarcoma patients. We report a case of a patient with metastatic pelvic osteosarcoma, who had been started on ipilimumab and nivolumab after failing treatment with doxorubicin, cisplatin, and regorafenib. He subsequently developed coffee-ground emesis 18 days after receiving ipilimumab and nivolumab and was found to have thrombocytopenia, anemia, elevated triglycerides, elevated ferritin, decreased soluble IL-2 receptor (IL2 R) levels, and decreased Natural Killer (NK) cell activity. Based on these criteria, a diagnosis of HLH secondary to immune checkpoint inhibitor use was made and the patient was discharged on a solumedrol taper while his cancer treatment was held. Unfortunately, the patient passed away approximately a month later due to progression of his disease.