Kikuchi-Fujimoto disease: Clinical and laboratory characteristics and outcome

Abstract

Introduction:Kikuchi-Fujimoto disease is an uncommon disorder with worldwide distribution, characterized by fever and benign enlargement of the lymph nodes, primarily affecting young adults. Awareness about this disorder may help prevent misdiagnosis and inappropriate investigations and treatment. The objective of the study was to evaluate the clinical and laboratory characteristics of histopathologically confirmed cases of Kikuchi's disease from a tertiary care center in southern India.Materials and Methods:Retrospective analysis of all adult patients with histopathologically confirmed Kikuchi's disease from January 2007 to December 2011 in a 2700-bed teaching hospital in South India was done. The clinical and laboratory characteristics and outcome were analyzed.Results:There were 22 histopathologically confirmed cases of Kikuchi's disease over the 5-year period of this study. The mean age of the subjects’ was 29.7 years (SD 8.11) and majority were women (Male: female- 1:3.4). Apart from enlarged cervical lymph nodes, prolonged fever was the most common presenting complaint (77.3%). The major laboratory features included anemia (54.5%), increased erythrocyte sedimentation rate (31.8%), elevated alanine aminotransferase (27.2%) and elevated lactate dehydrogenase (LDH) (31.8%).Conclusion:Even though rare, Kikuchi's disease should be considered in the differential diagnosis of young individuals, especially women, presenting with lymphadenopathy and prolonged fever. Establishing the diagnosis histopathologically is essential to avoid inappropriate investigations and therapy.

The incidence and clinical characteristics by gender differences in patients with Kikuchi–Fujimoto disease

“…Anemia was defined as having a hemoglobin level lower than 12 g/dL. [11] Thrombocytopenia was defined as a platelet count below 150,000 cells/μL. [19] Elevated ESR was defined as a level exceeding 60 mm/h.…”

“…[19] Elevated ESR was defined as a level exceeding 60 mm/h. [11] Elevated CRP was defined as a level exceeding 8 mg/L. [11] An abnormal LFT was defined as an alanine aminotransferase level exceeding 40 IU/L.…”

“…[8] KFD mainly occurs at a young age and has been reported to occur more frequently in women. [9–11] The male-to-female sex ratio varies from 1:16 to 1:4. [9–13] The sex distribution is almost equal in adult Asian populations, but among children aged 18 years or younger, male predominance has been reported (8:3).…”

Kikuchi-Fujimoto Disease: Unusual Presentation of Rare Disease

“…Histopathological findings include paracortical expansion with foci of coagulative necrosis and extensive karyorrhectic debris. 6 Several studies have shown CD8+ T lymphocytes and plasmacytoid dendritic cells as the predominant proliferating cells with a relative absence of neutrophils. 3,6,7 There are currently no treatment guidelines for KFD although spontaneous resolution typically occurs in one to six months with a low recurrence rate of 3-4%.…”

“…6 Several studies have shown CD8+ T lymphocytes and plasmacytoid dendritic cells as the predominant proliferating cells with a relative absence of neutrophils. 3,6,7 There are currently no treatment guidelines for KFD although spontaneous resolution typically occurs in one to six months with a low recurrence rate of 3-4%. 1,3,7 Due to its underlying inflammatory response, non-steroidal antiinflammatory drugs and antipyretics are important for symptomatic relief.…”