An Unusual Case of Hemophagocytic Lymphohistiocytosis Diagnosed by Spinal Nerve Root Biopsy

Authors

Leah Harburg, New York Medical College
Jared B. Cooper, New York Medical CollegeFollow
Allyson Flower, New York Medical CollegeFollow
Michael E. Tobias, New York Medical CollegeFollow
Avinash Mohan, New York Medical CollegeFollow

DOI

10.3171/2019.1.PEDS18591

Journal Title

Journal of Neurosurgery. Pediatrics

First Page

1

Last Page

5

Document Type

Case Report

Publication Date

April 2019

Department

Pediatrics

Abstract

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.

Recommended Citation

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