9y/o girl without systemic disease, was admitted due to right post. neck LAP with tenderness noted for 2 weeks. An episode of fever without obvious URI symptoms was noted 4 weeks ago, which lasted for 2 days. No family history of hematologic disease. No body weight loss, no night sweat, no URI symptoms recently and no skin rash. PE found bilateral tonsil enlargement (grade III) without exudate. LAP was limited to right post. neck, with tenderness, but no local heat nor erythema. Lab data showed normal in CBC, BCS. Operation of biopsy was perforemed today, that frozen showed "Kikuchi disease".
[Kikuchi disease] reference: eMedicine, Medscape
-1972年在日本由Kikuchi & Fujimoto兩位仁兄分別發表的.又稱necrotizing histiocytic lymphadenitis.
-臨床表現:
最常見是頸部淋巴腫大(80%)尤其是post. neck,無痛或是輕微壓痛;有些人有flu like prodrome, fever最常見,有些則是URI symptoms
少數人表現多處淋巴腫大或是皮膚的病灶(mobliform, maculopapular rash, molar rash, similar to SLE); 比較嚴重的還有肝脾腫大,無菌性腦膜炎,不過很少見.
大多數會自己好,淋巴腫大約持續數週~數月.癒後佳也沒什麼complication.
kikuchi disease的skin presentation. 來源: Dermatology Imgae Atlas.
-Pathophysiology:
未明,目前傾向與autoimmune有關,可能來自HLA type II or Tc cell activation.有genetic predisposition.
也有人提出跟virus infection的相關性(ex. CMV, EBV, HHV, VZV ),不過沒有證實.
-鑑別診斷:
malignant lymphoma, SLE, metastatic cancer with LN involvement.
-臨床治療:
等他自己好.有人提出跟SLE的相關性,不過尚未證實.
-病理特徵:(難!!!)
Extensive paracortical area of coagulative necrosis
Large lymphoid cells and immunoblasts, some of which have wide cytoplasm with an eccentric nucleus (arrow)
Karyorrhectic foci with large numbers of histiocytes, including crescentic histiocytes (arrow), and some lymphoid cells
組織學上分三種:
- Proliferative phase - Initial phase with typical findings noted above
- Necrotizing phase - Extensive necrosis that may destroy normal architecture of the node
- Xanthomatous ("foamy cell") phase - The recovery phase with resolution of necrosis
第二種表現最常見.最具特色的細胞是histiocytes & plasmacytoid monocytes
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所以病人沒事,可以回家了.~~~~
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