Kikuchi-Fujimoto Disease: A 15-Year Analysis at a Children's Hospital in the United States

University of Texas Southwestern Medical Center, Dallas, TX, USA
Clinical Pediatrics (Impact Factor: 1.15). 02/2012; 52(1). DOI: 10.1177/0009922811436338
Source: PubMed
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    • "In addition, for our search, those articles that did not provide the ethnicity, age, and gender of the patients studied were not included in our review. We focused our review on the adult population although it is known that KFD may also occur in children.6 A total of 22 cases with KFD have been fully described in the literature.7–24 "
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    ABSTRACT: Kikuchi-Fujimoto Disease (KFD), also known as histiocytic lymphadenitis, is a benign, self-limiting disease that manifests primarily as cervical lymphadenopathy but may include low-grade fever, headache, and fatigue. There is a higher incidence of KFD in women aged 20-35 years and in Asian populations. A PubMed search revealed 590 articles that described KFD. Of these, 22 cases have been fully described in the United States. Ten of the 22 (45%) patients were male and 12 (55%) were female, with 20% Caucasian, 20% Asian American, and the remaining 60% of other ethnic backgrounds. In this study, we describe an additional 3 cases of KFD and discuss the diagnosis, pathology, and management of KFD.
    Mediterranean Journal of Hematology and Infectious Diseases 01/2014; 6(1):e2014001. DOI:10.4084/MJHID.2014.001
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    ABSTRACT: A 38-year-old HIV-positive Nigerian woman presented with a three-week history of cervical lymphadenopathy, night sweats, weight loss and fever. Provisional diagnoses of tuberculosis and lymphoma were made; however, a histological diagnosis of Kikuchi-Fujimoto Disease was reached. This rare benign disease has presenting features that mimic more serious conditions commonly occurring in HIV-positive patients. This case report emphasises the importance of Kikuchi-Fujimoto Disease in the differential diagnosis of cervical lymphadenopathy in HIV-positive patients.
    International Journal of STD & AIDS 07/2014; 26(8). DOI:10.1177/0956462414545793 · 1.05 Impact Factor
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    ABSTRACT: Background: Kikuchi-Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. This disease is misdiagnosed as malignant lymphoma in up to one-third of cases and is associated with the development of systemic lupus erythematosus (SLE). Methods: The medical literature between the years 1972 and 2014 was searched for KFD, and the data were collected and analyzed regarding the epidemiology, clinical presentations, diagnosis, management, and suggested diagnostic and treatment algorithms. Results: Although KFD has been reported in other ethnic groups and geographical areas, it is more frequently diagnosed in young women of Asian descent. Patients with the disease typically present with rapidly evolving tender cervical lymphadenopathy, night sweats, fevers, and headache. Diagnosis is based on histopathological examination. Excisional lymph node biopsy is essential for a correct diagnosis. Apoptotic coagulation necrosis with karyorrhectic debris and the proliferation of histiocytes, plasmacytoid dendritic cells, and CD8(+) T cells in the absence of neutrophils are characteristic cytomorphology features. Interface dermatitis at the onset of KFD may be a marker for the subsequent evolution of SLE. The natural course of the disease is typically benign. Short courses of steroids, nonsteroidal anti-inflammatory drugs, or hydroxychloroquine can be administered to patients with more severe symptoms. Conclusions: Although KFD was described more than 40 years ago, the etiology of this disease remains unsolved. Infectious or autoimmune processes were proposed but have not been definitively confirmed. Clinical presentation with systemic B symptoms and adenopathy may lead to an erroneous diagnosis of malignant lymphoma. The introduction of modern methods into hematopathology, including immunohistochemistry, flow cytometry, and molecular clonality studies, has decreased the probability of misdiagnosis. Until reliable prognostic markers are available, patients with KFD should have continued long-term follow-up care due to their increased risk of SLE.
    Cancer control: journal of the Moffitt Cancer Center 10/2014; 21(4):313-321. · 3.50 Impact Factor
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