Persistent cervical lymphadenopathy in an adolescent with Epstein-Barr induced hemophagocytic syndrome: manifestations of a rare but often fatal disease.
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a non-malignant proliferative disorder characterized by histiocytic proliferation and hemophagocytosis following Epstein-Barr virus infection. Though quite rare, this condition represents an often fatal disease primarily affecting the pediatric population. We discuss the case of an adolescent female who presented initially with persistent cervical lymphadenopathy and the typical findings of tonsillar hypertrophy, pharyngitis, and splenomegaly associated with infectious mononucleosis. This case study outlines the pathogenesis, common clinical findings, diagnostic criteria, and a review of the HLH-94 treatment protocol. Early recognition and treatment is emphasized because of the fulminant course of the disorder.
Lindemann
Timothy L
TL
Geisinger Medical Center, Department of Otolaryngology/Head and Neck Surgery, M.C. 13-33, 100 N. Academy Avenue, Danville, PA 17822, USA. tllindemann@geisinger.edu
Greene
J Scott
JS
D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003907:Dexamethasone / Q000627:therapeutic use; D005047:Etoposide / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis / Q000821:virology*; D006801:Humans; D007244:Infectious Mononucleosis / Q000175:diagnosis* / Q000188:drug therapy; D008206:Lymphatic Diseases / Q000175:diagnosis / Q000821:virology*; D009333:Neck; D019384:Nucleic Acid Synthesis Inhibitors / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D019384:Nucleic Acid Synthesis Inhibitors; D005047:Etoposide; D003907:Dexamethasone