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Volume 43, Number 3, June 2010

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


Received: February 25, 2009    Revised: April 30, 2009    Accepted: June 19, 2009   

 

Corresponding author:

 Yhu-Chering Huang

 

Department of Pediatrics, Chang

Gung Memorial Hospital and College of Medicine, Chang

Gung University, 5 Fu-Shin Street, Kweishan, Taoyuan

County 333, Taiwan.

E-mail: ychuang@adm.cgmh.org.tw

 



 

Background and purpose: 

 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).