Abstract
Objectives
Kikuchi-Fujimoto disease (KFD) is characterized by lymphadenopathy and fever, and is usually self-limited. This study analyzed the clinical characteristics of pediatric patients with KFD.
Methods
This retrospective, observational, single-center study was conducted in South Korea from March 2008 to October 2015. KFD was diagnosed based on clinical, radiological or histological findings and excluded when there were any other causes of lymphadenopathy. Medical records were reviewed for clinical and laboratory manifestations.
Results
A total of 35 cases were included. The mean patient age was 12.1±2.9 years (range, 5 to 17 years); the male-to-female ratio was 1:0.8. The main clinical manifestations were cervical lymphadenopathy and fever in 34 cases (97%). The mean duration of fever was 12.2±8.3 days (range, 2 to 37 days). We noted enlargement of lymph nodes in the cervical, mesenteric (n=5, 14%), axillary (n=2, 6%), and inguinal (n=1, 3%) regions. Hepatosplenomegaly, loss of appetite, and rash were observed. On laboratory examinations, elevation of ferritin, leukopenia, and positivity for anti-nuclear antibodies were frequently observed. Twelve patients underwent biopsy and 23 cases were diagnosed by radiological findings. The mean duration of hospitalization for all cases was 7.9±2.9 days (range, 3 to 13 days) and steroids were administered in 10 cases. KFD recurrence was observed in 2 cases (5.7%) with the time to relapse of 7 months and 4 years. There were no cases with systemic lupus erythematous or other autoimmune disease.
Figures and Tables
Table 1
Values are presented as number of cases (%) or mean±SD (range) unless otherwise indicated.
*Not recorded in 13 cases.
†Accompanied by cervical lymphadenopathy except only one case of axillary lymphadenopathy.
‡The mesenteric lymph node involvement was examined by imaging studies (computed tomography or sonography) on 14 patients, five of them were detected.
References
1. Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytosis: a clinicopathological study. Acta Haematol Jpn. 1972; 35:379–380.
2. Fujimoto Y, Kojima Y, Yamaguchi K. Cervical subacute necrotizing lymphadenitis: a new clinicopathological entity. Intern Med. 1972; 20:920–927.
3. Kuo T, Shih LY. Surgical pathology of lymph node biopsy specimens in Taiwan with an update on adult T cell leukaemia/lymphoma. In : Hanaoka M, Kadin ME, Mikata A, editors. Lymphoid malignancy: immunocytology and cytogenetics. New York: Field and Wood;1990. p. 109–115.
5. Yoon SH, Song MH, Shin SH, Kim SK, Lee KH, Yoon HS, et al. A case of Kikuchi-Fujimoto disease, subsequently evolving to systemic lupus eyrthematosus. Korean J Pediatr. 2004; 47:904–907.
6. Martinez-Vazquez C, Hughes G, Bordon J, Alonso-Alonso J, Anibarro-Garcia A, Redondo-Martinez E, et al. Histiocytic necrotizing lymphadenitis, Kikuchi-Fujimoto's disease, associated with systemic lupus erythemotosus. QJM. 1997; 90:531–533.
7. Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M. Kikuchi-Fujimoto disease: analysis of 244 cases. Clin Rheumatol. 2007; 26:50–54.
8. Kikuchi M, Takeshita M, Eimoto T, Iwasaki H, Minamishima Y, Maedo Y. Histiocytic necrotizing lymphadenitis: clinicopathologic, immunologic and HLA typing study. In : Hanoaka M, Kadin ME, Mikata A, editors. Lymphoid malignancy: immunocytologic and cytogenetics. New York: Field & Wood Medical Publishers;1990. p. 251–257.
9. Kang HM, Kim JY, Choi EH, Lee HJ, Yun KW, Lee H. Clinical characteristics of severe histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) in children. J Pediatr. 2016; 171:208–212.
10. Hong JY, Bae SH, Kim WS. Clinical features of subacute necrotizing lymphadenitis in children. J Korean Pediatr Soc. 2002; 45:994–999.
11. Lin HC, Su CY, Huang SC. Kikuchi's disease in Asian children. Pediatrics. 2005; 115:e92–e96.
12. Seo JH, Shim HS, Park JJ, Jeon SY, Kim JP, Ahn SK, et al. A clinical study of histiocytic necrotizing lymphadenitis (Kikuchi's disease) in children. Int J Pediatr Otorhinolaryngol. 2008; 72:1637–1642.
13. Wang TJ, Yang YH, Lin YT, Chiang BL. Kikuchi-Fujimoto disease in children: clinical features and disease course. J Microbiol Immunol Infect. 2004; 37:219–224.
14. Park HS, Sung MJ, Park SE, Lim YT. Kikuchi-Fujimoto disease of 16 children in a single center of Korea. Pediatr Allergy Immunol. 2007; 18:174–178.
15. Jang JM, Woo CH, Choi JW, Song DJ, Yoo Y, Lee KC, et al. A case of Kikuchi's disease with skin involvement. Korean J Pediatr. 2006; 49:103–106.
16. Park SJ, Moon WJ, Kim WS, Kim KS. Kikuchi-Fujimoto disease with aseptic meningitis. Korean J Pediatr. 2009; 52:622–626.
18. Lee KY, Yeon YH, Lee BC. Kikuchi-Fujimoto disease with prolonged fever in children. Pediatrics. 2004; 114:e752–e756.
19. Dumas G, Prendki V, Haroche J, Amoura Z, Cacoub P, Galicier L, et al. Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature. Medicine (Baltimore). 2014; 93:372–382.
20. Deaver D, Horna P, Cualing H, Sokol L. Pathogenesis, diagnosis, and management of Kikuchi-Fujimoto disease. Cancer Control. 2014; 21:313–321.
21. Doh JH, Kim ST, Lee KS, Jeong SY. The lymphocyte subset change in a case of subacute necrotizing lymphadenitis. J Korean Pediatr Soc. 1998; 41:994–1000.
22. Kwon SY, Kim TK, Kim YS, Lee KY, Lee NJ, Seol HY. CT findings in Kikuchi disease: analysis of 96 cases. AJNR Am J Neuroradiol. 2004; 25:1099–1102.
23. Chung PS, Lee SJ, Yoon JS, Park SJ, Kim JS, Moon JH. Clinical and radiologic characteristics of Kikuchi's disease. Korean J Otolaryngol-Head Neck Surg. 2006; 49:85–89.
24. Chiang YC, Chen RM, Chao PZ, Yang TH, Lee FP. Pediatric Kikuchi-Fujimoto disease masquerading as a submandibular gland tumor. Int J Pediatr Otorhinolaryngol. 2004; 68:971–974.
25. Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol. 2004; 122:141–152.
26. Jang YJ, Park KH, Seok HJ. Management of Kikuchi's disease using glucocorticoid. J Laryngol Otol. 2000; 114:709–711.
27. Dorfman RF. Histiocytic necrotizing lymphadenitis of Kikuchi and Fujimoto. Arch Pathol Lab Med. 1987; 111:1026–1029.
28. O'Neill D, O'Grady J, Variend S. Child fatality associated with pathological features of histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease). Pediatr Pathol Lab Med. 1998; 18:79–88.
Supplementary Material
Supplementary Table is available from: https://doi.org/10.12771/emj.2018.41.4.75