Kikuchi‐Fujimoto disease of 16 children in a single center of Korea

Abstract

Kikuchi-Fujimoto disease (KFD) is subacute necrotizing lymphadenitis characterized by fever, leukopenia and cervical lymphadenopathy. There are few reports on the clinical characteristics and laboratory findings of KFD in the pediatric literature. In this study, we evaluate the characteristics and outcome of KFD in children. A total of 412 patients were studied with fever and peripheral lymphadenopathy at Pusan National University Hospital from January 1998 to December 2003. Among the total 412 there were 16 patients diagnosed with KFD by lymph node biopsy. We analyze the clinical, laboratory and outcome after review of the medical records retrospectively. The mean age of the patients was 10.6 +/- 3.4 yr (range: 4-17 yr). The male to female ratio was 1:1. Almost all patients, except two, had cervical lymph node swelling. The size of the involved lymph node was less than 4 cm in the greatest dimension in 75% of the patients. All the children had fever as the chief complaint and the mean duration of the fever was 17.7 +/- 11.2 days (range: 2-122 days). Approximately 87% of the patients had leukopenia (WBC < 4000/mm(3)) and 43.8% of the patients had a mild increase in the transaminases on liver function testing. A total of 8 out of the 16 patients were initially misdiagnosed as an infectious disease and treated with antibiotics which caused prolonged hospitalization for most patients. Six patients were treated with prednisone and the prolonged fever subsided immediately after steroid therapy. KFD should be considered in the differential diagnosis of prolonged fever in children with cervical lymphadenopathy. Early cervical lymph node biopsy is necessary to minimize inappropriate examinations and treatments in such cases.

Kikuchi-Fujimoto disease: three case reports

“…No studies have confirmed any positive serological findings relating to antinuclear antibodies, rheumatoid factors or other immunological parameters, as signs of the autoimmune nature of this disease. [1][2][3][9][10][11] Recent studies have demonstrated activated CD8+ cells after viral infection in lymph nodes, which may induce apoptosis of CD4+ lymphocytes. Hypothetically, apoptotic lymphocytes could deliver nuclear antigens and trigger autoimmune T and B cells to produce antinuclear antibodies.…”

“…6,7 The lymph nodes are mainly small (less than 3 cm), mobile and painless. 3,6,7 Fever (usually high) is the first symptom in 30% to 50% of the cases; weight loss is observed in 10% and shivering in 4%. 7,9,14,15 Other less common manifestations include skin rash, gastrointestinal abnormalities and night sweating.…”

“…[1][2][3] They studied patients treated for lymphoma who evolved surprisingly well, showing much faster recovery than expected. In fact, these patients did not have lymphoma but a condition that has been called Kikuchi-Fujimoto disease since its initial description.…”

“…5 The disease is benign and self-limiting. 3,6,7 Rheumatological diseases such as SLE and Sjögren's syndrome may be associated with KFD, and therefore, investigation of the presence of these diseases and patient follow-up are necessary. 8 The etiology of Kikuchi-Fujimoto disease is uncertain, and speculations exist regarding relationships with previous viral infections or autoimmune processes.…”

“…8 The etiology of Kikuchi-Fujimoto disease is uncertain, and speculations exist regarding relationships with previous viral infections or autoimmune processes. [1][2][3] We present three cases involving female patients of non-Asian origin with Kikuchi-Fujimoto disease who were seen between 2003 and 2006 at our outpatient department, along with a short review of the literature.…”