Clinicopathologic study of Kikuchi’s disease in children in a tertiary hospital in South India
Keywords:C- reactive protein, Erythrocyte sedimentation rate, Kikuchi-Fujimoto disease, Lactate dehydrogenase, Systemic lupus erythematosus
Background: The aim of this study was to evaluate the clinical and laboratory characteristics, treatment modalities and outcome of children with Kikuchi’s disease.
Methods: A retrospective cross-sectional study was conducted among all children, histopathologically diagnosed with KFD. Clinical, laboratory data and treatment outcomes were analysed.
Results: During the study period, 53 children histopathologically confirmed as KFD were enrolled in the study. There were 36 males and 17 females. The lymph node involvements were mostly cervical with bilateral predisposition (63.5%), firm (88%), matted (30.8%) and tenderness (38.5%). Fever, headache, vomiting, chills, myalgia and rash were other common presentations other than cervical lymphadenopathy. The associated laboratory findings include anemia (71.2%), leukopenia especially lymphopenia (31.4%), monocytosis (21.6%), thrombocytopenia (16.3%), elevated CRP (53.1%), ESR (83.7%), LDH (100%) and elevated liver enzymes. Most of the children were managed conservatively (49.1%). Corticosteroids were administered for (22.6 %) of patients. Recurrence occurred in 4 children (7.5 %) and 13 children (24.5%) had other associated diseases.
Conclusions: KFD should be suspected in well children with febrile cervical lymphadenopathy, especially with leukopenia, monocytosis, and elevated CRP, ESR, LDH, Liver enzymes. KFD in children can have rarely atypical presentations and coexist with other diseases.
Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytosis. Nippon Ketsueki Gakkai Zasshi. 1972;35:379-80.
Fujimoto Y, Kojima Y, Yamaguchi K. Cervical subacute necrotizing lymphadenitis. A new clinicopathological entity. New Clin Ent. 1972;20:920-7.
Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol. 2004 Jul;122(1):141-52.
Pepe F, Disma S, Teodoro C, Pepe P, Magro G. Kikuchi-Fujimoto disease: a clinicopathologic update. Pathologica. 2016;108(3):120-9.
Bosch X, Guilabert A. Kikuchi-Fujimoto disease. Orphanet J Rare Dis. 2006;1:18.
Lin YC, Huang HH, Nong BR, Liu PY, Chen YY, Huang YF, et al. Pediatric Kikuchi-Fujimoto disease: A clinicopathologic study and the therapeutic effects of hydroxychloroquine. J Microbio Immunol Infect. 2017 Sep 29.
Kim TY, Ha K-S, Kim Y, Lee J, Lee K, Lee J. Characteristics of Kikuchi-Fujimoto disease in children compared with adults. Eur J Pediatr. 2014;173(1):111-6.
Cheng C-Y, Sheng W-H, Lo Y-C, Chung C-S, Chen Y-C, Chang S-C. Clinical presentations, laboratory results and outcomes of patients with Kikuchi’s disease: emphasis on the association between recurrent Kikuchi’s disease and autoimmune diseases. J Microbiol Immunol Infect. 2010;43(5):366-71.
Kuo TT. Kikuchi’s disease (histiocytic necrotizing lymphadenitis). A clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and DNA ploidy. Am J Surg Pathol. 1995;19(7):798-809.
Rezayat T, Carroll MB, Ramsey BC, Smith A. A case of relapsing Kikuchi-Fujimoto disease. Case Reports Otolaryngol. 2013;2013.
Quintás-Cardama A, Fraga M, Cozzi SN, Caparrini A, Maceiras F, Forteza J. Fatal Kikuchi-Fujimoto disease: the lupus connection. Ann Hematol. 2003;82(3):186-8.
Zou CC, Zhao ZY, Liang L. Childhood Kikuchi-Fujimoto disease. Indian J Pediatr. 2009;76(9):959-62.
Chen CK, Low Y, Akhilesh M, Jacobsen AS. Kikuchi disease in Asian children. J Paediatr Child Health. 2006;42(3):104-7.
Song JY, Lee J, Park DW, Sohn JW, Suh SI, Kim IS, et al. Clinical outcome and predictive factors of recurrence among patients with Kikuchi’s disease. Int J Infectious Dis. 2009;13(3):322-6.
Deaver DD, Horna P, Cualing H, Sokol L. Pathogenesis, diagnosis, and management of Kikuchi-Fujimoto Disease. Cancer Control. 2014;21(4):313-21.
Rosado FGN, Tang Y-W, Hasserjian RP, McClain CM, Wang B, Mosse CA. Kikuchi-Fujimoto lymphadenitis: role of parvovirus B-19, Epstein-Barr virus, human herpesvirus 6, and human herpesvirus 8. Hum Pathol. 2013;44(2):255-9.
Cho M-S, Choi HJ, Park HK, Cho SE, Han WS, Yang WI. Questionable Role of Human Herpesviruses in the Pathogenesis of Kikuchi Disease. Arch Pathol Laborat Med. 2007;131(4):604-9.
Chong Y, Kang CS. Causative agents of Kikuchi-Fujimoto disease (histiocytic necrotizing lymphadenitis): a meta-analysis. Int J Pediatr Otorhinolaryngol. 2014;78(11):1890-7.
Sopeña B, Rivera A, Chamorro A, Freire M, Alende V, Seco E, et al. Clinical association between Kikuchi׳s disease and systemic lupus erythematosus: A systematic literature review. Semin Arthritis Rheum. 2017;47(1):46-52.
Yu HL, Lee SS, Tsai HC, Huang CK, Chen YS, Lin HH, et al. Clinical manifestations of Kikuchi's disease in southern Taiwan. J microbiology, Immunol Infection= Wei mian yu gan ran za zhi. 2005;38(1):35-40.
Lin H-C, Su C-Y, Huang C-C, Hwang C-F, Chien C-Y. Kikuchi’s disease: a review and analysis of 61 cases. Otolaryngol Head Neck Surg. 2003;128(5):650-3.
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(2):174-8.
Han HJ, Lim G-Y, Yeo D-M, Chung N-G. Kikuchi’s disease in children: clinical manifestations and imaging features. J Korean Med Sci. 2009;24(6):1105-9.
Lee K-Y, Yeon Y-H, Lee B-C. Kikuchi-Fujimoto disease with prolonged fever in children. Pediatr. 2004;114(6):e752-6.
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. Med. 2014;93(24).
Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M. Kikuchi–Fujimoto Disease: analysis of 244 cases. Clin Rheumatol. 2007;26(1):50-4.
Rakesh P, Alex RG, Varghese GM, Mathew P, David T, Manipadam MT, et al. Kikuchi-Fujimoto Disease: Clinical and Laboratory Characteristics and Outcome. J Glob Infect Dis. 2014;6(4):147-50.
Rammohan A, Cherukuri SD, Manimaran AB, Manohar RR, Naidu RM. Kikuchi-Fujimoto Disease: A Sheep in Wolf’s Clothing. J Otolaryngol - Head Neck Surgery. 2012;41(3):222-6.
Basu D, Mutha SM. Histiocytic necrotizing lymphadenitis (Kikuchi Fujimoto Disease)-a report of four cases. Indian J Pathol Microbiol. 2002;45(1):89-91.
Singhania P, Paul R, Maitra S, Banerjee A, Hashmi M. Kikuchi-Fujimoto Disease from Eastern India. J Glob Infect Dis. 2010;2(3):305-6.
Mohan A, Reddy MK, Phaneendra BV, Chandra A. Aetiology of peripheral lymphadenopathy in adults: analysis of 1724 cases seen at a tertiary care teaching hospital in southern India. Natl Med J India. 2007;20(2):78-0.
Atwater AR, Longley BJ, Aughenbaugh WD. Kikuchi’s disease: case report and systematic review of cutaneous and histopathologic presentations. J Am Acad Dermatol. 2008;59(1):130-6.
De Filipp Z. Cutaneous Manifestation of Kikuchi-Fujimoto Disease in the Setting of Granulomatosis with Polyangiitis (Wegener’s). J Gen Intern Med. 2012;27(9):1220-2.
Komagamine T, Nagashima T, Kojima M, Kokubun N, Nakamura T, Hashimoto K, et al. Recurrent aseptic meningitis in association with Kikuchi-Fujimoto disease: case report and literature review. BMC Neurol. 2012;12:112.
Chan JK, Wong KC, Ng CS. A fatal case of multicentric Kikuchi’s histiocytic necrotizing lymphadenitis. Cancer. 1989;63(9):1856-2.
Silva AF da, Focaccia R, Oliveira AC de, Sementilli A, Reis GFB. Kikuchi-Fujimoto disease: an unusual association with acute renal failure. Brazilian J Infectious Dis. 2010;14(6):621-7.
Lim G-Y, Cho B, Chung NG. Hemophagocytic lymphohistiocytosis preceded by Kikuchi disease in children. Pediatr Radiol. 2008;38(7):756-61.
Chen JS, Chang KC, Cheng CN, Tsai WH, Su IJ. Childhood hemophagocytic syndrome associated with Kikuchi’s disease. Haematologica. 2000;85(9):998-1000.
Hu S, Kuo TT, Hong HS. Lupus lymphadenitis simulating Kikuchi's lymphadenitis in patients with systemic lupus erythematosus: a clinicopathological analysis of six cases and review of the literature. Pathology international. 2003;53(4):221-6.
Sykes JA, Badizadegan K, Gordon P, Sokol D, Escoto M, Ten I, et al. Simultaneous Acquired Self-limited Hemophagocytic Lymphohistiocytosis and Kikuchi Necrotizing Lymphadenitis in a 16-Year-Old Teenage Girl: A Case Report and Review of the Literature. Pediatr Emerg Care. 2016;32(11):792-8.