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

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

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
Volume43
Issue number3
DOIs
Publication statusPublished - Jun 2010
Externally publishedYes

Fingerprint

Histiocytic Necrotizing Lymphadenitis
Hemophagocytic Lymphohistiocytosis
Epstein-Barr Virus Infections
Axilla
Intravenous Immunoglobulins
Splenomegaly
Etoposide
Serology
Ferritins
Exanthema
Abdomen
Pregnant Women
Fever
Neck
Extremities
Bone Marrow
Steroids
Biopsy
Recurrence
Skin

Keywords

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

ASJC Scopus subject areas

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

Cite this

Primary Epstein-Barr Virus Infection Associated with Kikuchi's Disease and Hemophagocytic Lymphohistiocytosis : A Case Report and Review of the Literature. / Lee, Hao Yuan; Huang, Yhu Chering; Lin, Tzou Yien; Huang, Jing Long; Yang, Chao Ping; Hsueh, Tsun; Wu, Chang Teng; Hsia, Shao Hsuan.

In: Journal of Microbiology, Immunology and Infection, Vol. 43, No. 3, 06.2010, p. 253-257.

Research output: Contribution to journalArticle

Lee, Hao Yuan ; Huang, Yhu Chering ; Lin, Tzou Yien ; Huang, Jing Long ; Yang, Chao Ping ; Hsueh, Tsun ; Wu, Chang Teng ; Hsia, Shao Hsuan. / Primary Epstein-Barr Virus Infection Associated with Kikuchi's Disease and Hemophagocytic Lymphohistiocytosis : A Case Report and Review of the Literature. In: Journal of Microbiology, Immunology and Infection. 2010 ; Vol. 43, No. 3. pp. 253-257.
@article{847c98b624a545f98b876cc7d25bbe24,
title = "Primary Epstein-Barr Virus Infection Associated with Kikuchi's Disease and Hemophagocytic Lymphohistiocytosis: A Case Report and Review of the Literature",
abstract = "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).",
keywords = "Epstein-Barr virus, hemophagocytic lymphohistiocytosis, Kikuchi's disease",
author = "Lee, {Hao Yuan} and Huang, {Yhu Chering} and Lin, {Tzou Yien} and Huang, {Jing Long} and Yang, {Chao Ping} and Tsun Hsueh and Wu, {Chang Teng} and Hsia, {Shao Hsuan}",
year = "2010",
month = "6",
doi = "10.1016/S1684-1182(10)60040-0",
language = "English",
volume = "43",
pages = "253--257",
journal = "Journal of Microbiology, Immunology and Infection",
issn = "0253-2662",
publisher = "Elsevier Taiwan LLC",
number = "3",

}

TY - JOUR

T1 - Primary Epstein-Barr Virus Infection Associated with Kikuchi's Disease and Hemophagocytic Lymphohistiocytosis

T2 - A Case Report and Review of the Literature

AU - Lee, Hao Yuan

AU - Huang, Yhu Chering

AU - Lin, Tzou Yien

AU - Huang, Jing Long

AU - Yang, Chao Ping

AU - Hsueh, Tsun

AU - Wu, Chang Teng

AU - Hsia, Shao Hsuan

PY - 2010/6

Y1 - 2010/6

N2 - 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).

AB - 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).

KW - Epstein-Barr virus

KW - hemophagocytic lymphohistiocytosis

KW - Kikuchi's disease

UR - http://www.scopus.com/inward/record.url?scp=77953111101&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77953111101&partnerID=8YFLogxK

U2 - 10.1016/S1684-1182(10)60040-0

DO - 10.1016/S1684-1182(10)60040-0

M3 - Article

VL - 43

SP - 253

EP - 257

JO - Journal of Microbiology, Immunology and Infection

JF - Journal of Microbiology, Immunology and Infection

SN - 0253-2662

IS - 3

ER -