A 41‐year‐old African American woman presented with a 3‐week history of low‐grade fever, malaise, generalized arthralgia, unintentional 20‐pound weight loss, and tender left posterior cervical lymphadenopathy. She reported no animal exposure, international travel, illicit drug use, or exposure to tuberculosis. Physical exam confirmed firm and tender 2/2 cm left posterior cervical lymph nodes. Evaluation showed anemia (hemoglobin 7 g/dL, MCV 67), leukopenia, and elevated inflammatory markers (erythrocyte sedimentation rate and C‐reactive protein). The rest of the lymphoreticular system examination was within normal limits. A CT neck confirmed left posterior cervical lymph node enlargement with features suggestive of reactive lymphadenitis. A further workup was warranted for localized lymphadenopathy with mononucleosis‐like syndrome, as she had an atypical and protracted presentation. Serologies for viral (CMV, EBV, HIV), bacterial, and autoimmune [systemic lupus erythematosus (SLE)] causes were negative. An excision biopsy of the lymph nodes revealed subtotal necrosis of the lymph node with features consistent with histiocytic necrotizing lymphadenopathy (Kikuchi–Fujimoto disease).
Kikuchi lymphadenitis was first described in 1972 as a rare cause of lymphadenopathy of unclear etiology and pathogenesis. It typically manifests as posterior cervical lymphadenopathy, low‐grade fever, and rarely with hepatosplenomegaly, weight loss, and night sweats, mimicking connective tissue diseases, atypical infections, and lymphoid malignancies. It typically is described in younger Asians but is being recognized in both sexes and across all ethnic groups. Although no specific laboratory test confirms the diagnosis, most patients have associated anemia, elevated inflammatory markers, and leukopenia. Most patients undergo an extensive workup for infectious, malignant, and autoimmune causes. Diagnosis often requires histopathologic examination of an excised lymph node with typical morphologic features (patchy distribution and abundance of karyorrhectic debris) and immunohistochemistry features (many CD68 + plasmocytoid dendritic cells). It is a self‐limiting condition with spontaneous recovery within months. Corticosteroids and hydroxychloroquine are reserved for severe and relapsing cases. SLE is an infrequent but commonly recognized association, and it becomes prudent to have periodic surveillance for these patients.
Kikuchi–Fujimoto disease is rare but is increasingly being recognized in the United States (more common in whites). It is often mistaken for a lymphoma, autoimmune lymphoproliferative syndromes, and IgG4‐related sclerosing disease; increased awareness with early recognition can prevent laborious investigations and inappropriate treatment. An early excision biopsy with morphological and immunohistochemical features confirmed by an experienced pathologist can expedite the diagnosis.
A. Cheema ‐ none; N. Upalakalin ‐ none; N. Kalva ‐ none
To cite this abstract:Cheema A, Upalakalin N, Kalva N. Not All Fever with Lymphadenopathy Is of Infectious, Malignant, or Autoimmune Etiology. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 249. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/not-all-fever-with-lymphadenopathy-is-of-infectious-malignant-or-autoimmune-etiology/. Accessed May 26, 2019.