Primary Epstein-Barr Virus Infection Associated with Kikuchi's Disease and Hemophagocytic Lymphohistiocytosis: A Case Report and Review of the Literature

Hao Yuan Lee, Yhu Chering Huang, Tzou Yien Lin, Jing Long Huang, Chao Ping Yang, Tsun Hsueh, Chang Teng Wu, Shao Hsuan Hsia

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20 Citations (Scopus)


An association between hemophagocytic lymphohistiocytosis (HLH) and Kikuchi's disease is rarely seen in children. Here, we present the case of a male adolescent (age 16 years and 3 months) who suffered from spiking fever for more than 1 week, and multiple nodules over the neck and bilateral axilla for 2 months. A skin rash also developed over all four limbs, abdomen and face. Laboratory data and skin biopsy gave results compatible with a diagnosis of Kikuchi's disease. Hemophagocytosis in the bone marrow, hyper-triglyceridemia, elevated ferritin, and splenomegaly were also found, fulfilling the criteria for HLH. A recent primary Epstein-Barr virus infection was also diagnosed by serology. The patient ran a relatively benign course. Intravenous immunoglobulins, steroids or etoposide-containing regimens were not used, and his recovery was uneventful. A review of the literature showed that up to February 2009, 11 additional cases of Kikuchi's disease presenting simultaneously with, or mimicking, HLH had been reported. Complete resolution within several weeks, with no recurrence, was seen in all but one patient (a pregnant woman).

Original languageEnglish
Pages (from-to)253-257
Number of pages5
JournalJournal of Microbiology, Immunology and Infection
Issue number3
Publication statusPublished - Jun 2010
Externally publishedYes



  • Epstein-Barr virus
  • hemophagocytic lymphohistiocytosis
  • Kikuchi's disease

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology and Microbiology(all)
  • Microbiology (medical)
  • Infectious Diseases

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