NYMC Faculty Publications

Title

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

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.

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