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Association Between Clinical Characteristics and Pathological Features of Kikuchi Fujimoto Disease in Department of Anatomical Pathology Cipto Mangunkusumo General Hospital.

Background: Kikuchi Fujimoto Disease (KFD) is a syndrome characterized by regional lymphadenopathy, benign, and accompanied by fever, also self-limiting. KFD is often misdiagnosed as tuberculosis, autoimmune disease, and malignancy because of the similarity of symptoms and histopathological features. A number of studies show the prevalence of KFD in Asia, including in Indonesia. The combination of clinical characteristics and microscopic evaluation is helpful for diagnosing and determining the prognosis of KFD. Until now not much is known about the characteristics and epidemiology of FCF in Indonesia. This study aims to determine the clinicopathological characteristics of KFD in Cipto Mangunkusumo General Hospital and to determine the association between clinical characteristics and histopathological features.Method: This study is a retrospective analytic cross-sectional study. The sample was taken from the medical records of patients with KFD at the Department of Anatomical Pathology Cipto Mangunkusumo General Hospital in 2016-2020. The data collected includes age, gender, location of lymphadenopathy, clinical diagnosis, clinical symptoms, lymph node size, number of lymph nodes involved, histopathology and immunohistochemical examination. The sampling method used is consecutive sampling. Data were analyzed using chi square and fisher exact test. Result: From 65 KFD samples, it was found that it mainly occurred in adult women, with age ranging from 19 - 59 years old, with lymphadenopathy in the neck area, < 2cm in size and solitary. The main histopathological appearance is phagocytic, necrotic, with < 50% area of coagulative necrosis. Ki67 expression was found in 43 samples with an area of < 30%. In the comparison between clinical characteristics and histopathological features, it was found that the area of coagulative necrosis of > 50% was found in cervical lymphadenopathy (p=0.000); Coagulative necrosis area of < 50% was found in lymph nodes of < 2cm (p=0.006). Histological subtypes were also significantly different with the location of lymphadenopathy (p=0.014), whereas lymphohistiocytic, phagocytic, necrotic and xanthomatous subtypes were found in cervical lymphadenopathy.Conclusion: KFD patients in RSCM have clinical characteristics in the form of adult women, ranging in age from 19 - 59 years with cervical lymphadenopathy. Clinical characteristics related to the location and size of lymphadenopathy showed significant histopathological differences in the area of necrosis and histological subtype. Further research needs to be done to determine the molecular characteristics related to the histopathological picture, which can be used as biomarkers of KFD.
Keywords: Kikuchi Fujimoto Disease, clinical characteristics, histopathology, lymphadenopathy


Latar Belakang: Kikuchi Fujimoto Disease (KFD) adalah suatu kelainan yang ditandai dengan limfadenopati regional, jinak, dan disertai demam, juga dapat sembuh sendiri. KFD sering salah didiagnosis sebagai tuberkulosis, penyakit autoimun, dan keganasan karena kemiripan gejala dan gambaran histopatologiknya. Sejumlah penelitian menunjukkan adanya prevalensi KFD di Asia termasuk di Indonesia. Kombinasi karakteristik klinis dan evaluasi mikroskopis sangat membantu untuk mendiagnosis dan menentukan prognosis KFD. Hingga saat ini tidak banyak diketahui tentang karakteristik dan epidemiologi KFD di Indonesia. Penelitian ini bertujuan untuk mengetahui karakteristik klinikopatologik KFD di RSUPN Cipto Mangunkusumo serta untuk mengetahui asosiasi antara karakteristik klinis dan gambaran histopatologisnya. Metode: Penelitian ini merupakan studi cross-sectional retrospektif analitik. Sampel penelitian ini diambil dari rekam medis pasien penderita KFD di Bagian Patologi Anatomi RSUPN Cipto Mangunkusumo tahun 2016-2020. Data yang dikumpulkan meliputi umur, jenis kelamin, lokasi limfadenopati, diagnosis klinis, gejala klinis, ukuran kelenjar getah bening, jumlah kelenjar getah bening yang terlibat, histopatologi dan pemeriksaan imunohistokimia. Metode pengambilan sampel yang digunakan adalah consecutive sampling. Data dianalisis menggunakan metode chi square dan fisher exact test. Hasil: Dari 65 sampel KFD, didapatkan bahwa terutama terjadi pada perempuan dewasa, rentang usia 19 - 59 tahun, dengan limfadenopati di daerah leher, ukuran < 2cm dan soliter. Gambaran histopatologis utamanya adalah fagositik, nekrotik, dengan area nekrosis koagulatif < 50%. Ekspresi Ki67 ditemukan pada 43 sampel dengan luas < 30%. Komparasi antara karakteristik klinis dan gambaran histopatologi, didapatkan bahwa luas nekrosis koagulatif > 50% ditemukan pada lokasi limfadenopati servikal (p=0,000); luas nekrosis koagulatif < 50% ditemukan pada kelenjar getah bening berukuran < 2cm (p=0,006). Subtipe histologis juga berbeda bermakna dengan lokasi limfadenopati (p=0,014), dimana subtipe limfohistiositik, phagocytic, necrotic dan xanthomatous ditemukan pada limfadenopati servikal. Kesimpulan: Pasien KFD di RSCM mempunyai karakteristik klinis berupa wanita dewasa, rentang usia 19 - 59 tahun dengan limfadenopati servikal. Karakteristik klinis terkait lokasi dan ukuran limfadenopati menunjukkan adanya perbedaan histopatologis bermakna dalam luas area nekrosis dan juga subtipe histologis. Penelitian lanjutan perlu dilakukan untuk mengetahui karakteristik molekuler terkait gambaran histopatologinya, yang dapat dijadikan sebagai biomarker KFD.
Kata kunci: Kikuchi Fujimoto Disease, karakteristik klinis, histopatologi, limfadenopati