Skip Navigation LinksHome > August 2012 - Volume 34 - Issue 6 > HHV-8-related Hemophagocytic Lymphohistiocytosis in a Boy Wi...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e3182375372
Clinical and Laboratory Observations

HHV-8-related Hemophagocytic Lymphohistiocytosis in a Boy With XLP Phenotype

Pasic, Srdjan MD, PhD*; Cupic, Maja MD; Lazarevic, Ivana MD

Collapse Box

Abstract

We report a 2.5-year-old boy with an X-linked lymphoproliferative disease (XLP) phenotype who presented with human herpes virus-8 (HHV-8)-related hemophagocytic lymphohistiocytosis (HLH). XLP is a rare primary immunodeficiency characterized by extreme susceptibility to herpes viruses, mainly Epstein-Barr virus (EBV). Approximately 60% of patients with XLP present with fulminant mononucleosis associated with HLH, whereas remaining patients present with hypogammaglobulinemia or lymphoproliferative disease. Most commonly, one of the XLP phenotypes appears after exposure to EBV, but at least 12% of affected individuals developed symptoms without an evidence of EBV infection. Rarely, patients with XLP may present with central nervous system vasculitis or aplastic anemia. HHV-8 is lymphotrophic and it is associated with lymphoproliferative disorders and Kaposi sarcoma in immunodeficient hosts. Kaposi sarcoma rarely occurs in children with well-defined primary immunodeficiency. Also, HHV-8-related HLH was previously reported in 2 siblings with a perforin gene deficiency. Recently, it became evident that besides EBV, other viruses may trigger the symptoms in XLP. We report for the first time HHV-8-related HLH in EBV-negative pediatric patient with an XLP phenotype.

© 2012 Lippincott Williams & Wilkins, Inc.

Login

Article Tools

Share

Search for Similar Articles
You may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search.

Connect With Us

Twitter
twitter.com/JPHOonline

For additional oncology content, visit LWW Oncology Journals on Facebook.