Kikuchi Fujimotova bolezen
Kikuchi-Fujimoto disease

slovenian

2008

volume 77, issue 1

diagnosis   Diagnostika   Histiocytic Necrotizing Lymphadenitis   Signs and Symptoms   therapy

Slovensko zdravniško društvo
(Obvezni izvod spletne publikacije)

Background. The Kikuchi Fujimoto disease (KFD) or histiocytic necrotising lymphadenitis is a rare benign disease, more common in Japanese women. In other parts of the world and also in Europe the disease in rare. The pathogenesis is poorly understood. Patients and methods. We represent 2 patients who observed enlarged peripheral nodes, measuring up to 2 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 the last two years, while in the second patient the mediastinal tumor mass became in 2 months larger. Another histological examination of the mass was performed and diffuse large anaplastic 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 mobile and measuring up to 2 cm. The only way to prove the disease is a histological examination of the node, which may show typical changes for the KFD: paracortical necrotic foci, surrounded by histiocytic aggregates, irregular rounded eosinophilic areas of different sizes in paracortex and cortex with the presence of numerous histiocytes, lymphocytes, immunoblasts, plasmacytoid monocytes and eosinophilic granulated cellular debris. It is typical not to find epithelioid cells and neutrophil granulocytes in the necrotic centres. The clinical picture of KFD is nonspecific and variable. The disease has to be distinguished especially from high grade lymphoma and SLE lymphadenitis. There is no specific medical treatment for KFD since the disease usually resolves spontaneously.

16.09.2009