Hemophagocytic lymphohistiocytosis preceded by Kikuchi disease in children
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Kikuchi disease (KD) is a type of benign, self-limiting lymphadenitis, but it has also been associated with hemophagocytic lymphohistiocytosis (HLH). To date, only a few reports have suggested an association between HLH and KD.
To report the imaging findings and clinical characteristics of KD accompanied by HLH in children.
Materials and methods
Five children with a prolonged fever and cervical lymphadenopathy were diagnosed as having HLH accompanied by KD. The authors retrospectively analyzed the clinical characteristics and the imaging findings in these children. The histology of excision biopsy samples of cervical lymph nodes in all children confirmed the diagnosis of KD. HLH was confirmed by bone marrow biopsy and laboratory criteria provided by the Histiocyte Society.
The greatest dimension of the enlarged nodes ranged from 0.5 cm to 2.5 cm and the nodes were most frequently located at level V. CT scans visualized perinodal infiltrates in most of the affected cervical nodes (four of five children) and extracervical nodes (three of three children). On enhanced CT scans, nonenhancing necrosis within the affected cervical nodes was noted in three children.
KD might be related to HLH in children. Systemic evaluations and follow-up of children with KD might help to identify HLH related to KD.
- Lee, KY, Yeon, YH, Lee, BC (2004) Kikuchi-Fujimoto disease with prolonged fever in children. Pediatrics 114: pp. 752-756 CrossRef
- Lin, HC, Su, CY, Huang, SC (2005) Kikuchi’s disease in Asian children. Pediatrics 115: pp. e92-e96 CrossRef
- Fitzgerald, NE, MacClain, KL (2003) Imaging characteristics of hemophagocytic lymphohistiocytosis. Pediatr Radiol 33: pp. 392-401
- Lindemann, TL, Greene, JC (2005) Persistent cervical lymphadenopathy in an adolescent with Epstein-Barr induced hemophagocytic syndrome: manifestations or a rare but often fatal disease. Int J Pediatr Otorhinolaryngol 69: pp. 1011-1014
- Lin, YW, Horiuchi, H, Ueda, I (2007) Recurrent hemophagocytic lymphohistiocytosis accompanied by Kikuchi’s disease. Leuk Lymphoma 48: pp. 2447-2451 CrossRef
- Mahadeva, U, Allport, T, Bain, B (2000) Haemophagocytic syndrome and histiocytic necrotizing lymphadenitis (Kikuchi’s disease). J Clin Pathol 53: pp. 636-638 CrossRef
- Khan, FY, Morad, NA, Fawzy, Z (2007) Kikuchi’s disease associated with hemophagocytosis. Chang Gung Med J 30: pp. 370-373
- Kim, YM, Lee, YJ, Nam, SO (2003) Hemophagocytic syndrome associated with Kikuchi’s disease. J Korean Med Sci 18: pp. 592-594 CrossRef
- Chen, JS, Chang, KC, Cheng, CN (2000) Childhood hemophagocytic syndrome associated with Kikuchi’s disease. Hemagologica 85: pp. 998-1000
- Gerritsen, A, Lam, K, Schneider, ME (2006) An exclusive case of juvenile myelomonocytic leukemia in association with Kikuchi’s disease and hemophagocytic lymphohistiocytosis and a review of the literature. Leuk Res 30: pp. 1299-1303 CrossRef
- Kwon, SY, Kim, TK, Kim, YS (2004) Findings in Kikuchi disease: analysis of 96 cases. AJNR 25: pp. 1099-1102
- Kelly, J, Kelleher, K, Khan, MK (2000) A case of haemophagocytic syndrome and Kikuchi-Fujimoto disease occurring concurrently in a 17-year-old female. Int J Clin Pract 54: pp. 547-549
- Hemophagocytic lymphohistiocytosis preceded by Kikuchi disease in children
Volume 38, Issue 7 , pp 756-761
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