A 33-year-old African American woman with no medical comorbidities and a recent trip to Jamaica presented to the clinic with complaints of multiple right-sided neck swellings. She reported developing laryngitis while she was in Jamaica, following which she noticed a right preauricular and a right submandibular mass that progressively increased in size. She also complained of fatigue, malaise, and intermittent chills. She had an episode of bilateral cervical lymphadenopathy 3 years earlier that spontaneously resolved. She had no prior history of tuberculosis or contact with it. She denied any history of smoking tobacco, drinking alcohol, or using illicit drugs. She denied taking any herbal or nonprescription medications at her house.
Physical examination revealed a comfortable and well-built young woman with anterior cervical, preauricular, submental, and right supraclavicular lymphadenopathy. The lymph nodes were firm and nontender. There was no hepatosplenomegaly, scleral icterus, or clinically appreciable lymphadenopathy elsewhere. Laboratory studies were significant for leucopenia with a total leucocyte count of 3200/muL and an ANC (absolute neutrophil count) of 700/muL. Patient was not anemic, with hemoglobin of 12.6 g/dL. Patient also had transaminitis with alanine aminotransferase of 116 U/L and aspartate aminotransferase of 64 U/L. Antinuclear antibody (ANA), rheumatoid factor (RF), and anti-DNA antibody were negative. Complement levels were within normal limits. Hepatitis-B surface antigen and neutrophil antibody assay were negative.
Chest X-ray was unremarkable. As the presentation was suspicious of lymphoma, a CT (computerized tomography) scan of neck, chest, abdomen, and pelvis was performed. It revealed asymmetric lymphadenopathy in the neck involving lymph nodes within the right posterior triangle (size of 2.8 cm), right internal jugular chain (size of 1.5 cm), bilateral submental lymph nodes (size of 1.2 cm), and right supraclavicular chain (size of 2 cm) (Figure 1). A fine needle aspiration biopsy of the lymph node showed mixed population of cells suggestive of a reactive process. An excisional biopsy of lymph node showed reactive follicles, an expanded parafollicular area (Figure 2) with clusters of large immunoblast-like cells, and histiocytes. Multiple areas showed necrotic foci with apoptotic debris (Figure 3). No granulocytes were present within these foci. Immunohistochemical staining showed CD3 positive T lymphocytes and CD20 positive B lymphocytes within the paracortical areas. Remnants of residual follicular dendritic cell meshworks and increased plasmacytoid dendritic cells were also found within the follicles. In situ hybridization for Ebstein-Barr virus encoded mRNA (EBER) was negative. These morphologic and immunophenotypic findings were suggestive of Kikuchi Fujimotos disease.
Patient was treated symptomatically with decrease in size of the lymph nodes gradually over the successive few weeks. Patient also experienced improvement in her fatigue and malaise. ANC improved to 1100/muL. Her prior self-remitted episode of bilateral cervical lymphadenopathy 3 years prior was likely the first episode of KFD and the current presentation a possible relapse.