KIKUCHI-FUJIMOTO DISEASE

Abstract

BACKGROUND The Kikuchi Fujimoto disease (KFD) or histiocytic necrotising lymphadenitis is a rarebenign disease, more common in Japanese women. In other parts of the world and also inEurope the disease in rare. The pathogenesis is poorly understood.Patients and methods We represent 2 patients who observed enlarged peripheral nodes, measuring up to2 cm. The second patient had also big mediastinal tumor mass. On histological examination of a peripheral node in both patients KFD was diagnosed. In both no medical treatment was prescribed. The first patient has been followed up without any symptoms for thelast two years, while in the second patient the mediastinal tumor mass became in 2 monthslarger. Another histological examination of the mass was performed and diffuse largeanaplastic CD 20 + lymphoma was diagnosed. CONCLUSIONS Most patients complain about enlarged cervical nodes accompanied by various symptoms,however the enlarged nodes rarely appear at other localizations. The nodes are mobileand measuring up to 2 cm. The only way to prove the disease is a histological examinationof the node, which may show typical changes for the KFD: paracortical necrotic foci,surrounded by histiocytic aggregates, irregular rounded eosinophilic areas of differentsizes in paracortex and cortex with the presence of numerous histiocytes, lymphocytes,immunoblasts, plasmacytoid monocytes and eosinophilic granulated cellular debris. It istypical not to find epithelioid cells and neutrophil granulocytes in the necrotic centres. Theclinical picture of KFD is nonspecific and variable. The disease has to be distinguishedespecially from high grade lymphoma and SLE lymphadenitis. There is no specific medicaltreatment for KFD since the disease usually resolves spontaneously