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Kikuchi-Fujimoto Disease (aka histiocytic necrotizing lymphadenitis) is a form of necrotizing lymphadenitis. The clinical presentation ranges from acute to subacute (2-3 week period) with painful unilateral cervical lymphadenopathy. Low grade fever, respiratory symptoms and other non-specific systemic symptoms are variable. Histologically, paracortical coagulative necrosis with abundant karyorrhectic debris is seen. These necrotic foci contain mainly histiocytes and so called plasmacytoid monocytes with a greater or lesser degree of lymphocytes and immunoblasts. Crescentic histiocytes are characteristic. Also characteristic is the absence of neutrophils in the areas of necrosis. This disease has many similarities with lupus lymphadenitis and there is much clinical and histologic overlap between the two entities.
Bosch et al. Am J Clin Pathol 2004;122:141-152
Note: The plasmacytoid monocytes seen in abundance in this condition are the proposed cell of origin for the CD4/CD56 hematodermic neoplasm aka NK blastic lymphoma
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