Pathogenesis, diagnosis, and management of Kikuchi-Fujimoto disease.

摘要: Background: Kikuchi–Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. This misdiagnosed as malignant lymphoma in up to one-third of cases and associated the development systemic lupus erythematosus (SLE). Methods: The medical literature between years 1972 2014 was searched for KFD, data were collected analyzed regarding epidemiology, clinical presentations, diagnosis, management, suggested diagnostic treatment algorithms. Results: Although KFD has been reported other ethnic groups geographical areas, it more frequently diagnosed young women Asian descent. Patients typically present rapidly evolving tender cervical lymphadenopathy, night sweats, fevers, headache. Diagnosis based on histopathological examination. Excisional lymph node biopsy essential correct diagnosis. Apoptotic coagulation necrosis karyorrhectic debris proliferation histiocytes, plasmacytoid dendritic cells, CD8 + T cells absence neutrophils are characteristic cytomorphology features. Interface dermatitis at onset may be marker subsequent evolution SLE. natural course benign. Short courses steroids, nonsteroidal anti-inflammatory drugs, or hydroxychloroquine can administered patients severe symptoms. Conclusions: described than 40 ago, etiology this remains unsolved. Infectious autoimmune processes proposed but have not definitively confirmed. Clinical presentation B symptoms adenopathy lead erroneous diagnosis lymphoma. introduction modern methods into hematopathology, including immunohistochemistry, flow cytometry, molecular clonality studies, decreased probability misdiagnosis. Until reliable prognostic markers available, should continued long-term follow-up care due their increased risk