12 July 2007 Ė Case of the Week #89
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Case of the Week #89
A 22 year old Caucasian woman presented with right cervical adenopathy.† Her medical history was otherwise unremarkable.† A lymph node was excised.
AFB and GMS stains were negative for fungi or microorganisms.† Serology was negative for toxoplasmosis, EBV or cat-scratch disease.
What is your diagnosis?
This disorder, also called histiocytic necrotizing lymphadenitis and Kikuchiís disease, was initially described in Japan and other Asian countries, but now is found worldwide.† It primarily affects young women, who present with cervical lymphadenopathy (tender or painless), fever and often leukopenia.† The etiology is unknown, although the prominent necrosis is due to apoptosis mediated by cytotoxic T cells (Mod Path 1997;10:231)
The microscopic picture is dominated by well circumscribed necrotic lesions, often with a starry sky appearance.† There is also karryorhexis, fibrin deposits, phagocytic and foamy histiocytes, plasmacytoid monocytes (two to three times larger than small lymphocytes with variable cytoplasm, round nuclei with open chromatin, small nucleoli, CD4+) and T cells (CD8+, cytotoxic phenotype).† There are no/rare plasma cells, neutrophils, follicular hyperplasia or atypia.† Occasionally, there is no overt necrosis (AJSP 1990;14:514).† Fine needle aspiration shows phagocytic histiocytes with peripheral nuclei and plasmacytoid monocytes.
The differential diagnosis includes lymphoma with necrosis, cat-scratch disease and lupus lymphadenitis.† Lymphoma may have prominent necrosis, but atypical cells should be present, and plasmacytoid monocytes are lacking.† Cat-scratch disease, caused by Bartonella henselae, has a clinical history of cat exposure with an initial skin papule.† Lymph nodes show prominent neutrophils and follicular hyperplasia, not present in Kukuchiís disease.† Lupus lymphadenitis has paracortical necrosis and hematoxylin bodies, as well as a clinical history of lupus (Hum Path 1989;20:295).
Nat Pernick, M.D., President
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