An Unusual Case of Hemophagocytic Lymphohistiocytosis Diagnosed by Spinal Nerve Root Biopsy
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease process characterized by aberrant immune system activation and an exaggerated inflammatory response. Establishing the diagnosis may be challenging and is achieved by satisfying a number of clinical criteria, in addition to demonstrating tissue hemophagocytosis. This syndrome is rapidly fatal if prompt diagnosis and treatment are not achieved. The authors present the case of a 17-year-old male patient with CNS HLH involving both the brain and spinal cord, highlighting the variable CNS manifestations in pediatric patients with HLH and the challenges that accompany establishing diagnosis.
Harburg, L., Cooper, J., Flower, A., Tobias, M., & Mohan, A. (2019). An Unusual Case of Hemophagocytic Lymphohistiocytosis Diagnosed by Spinal Nerve Root Biopsy. Journal of Neurosurgery. Pediatrics, 1-5. https://doi.org/10.3171/2019.1.PEDS18591