Kikuchi Disease Associated with Hemophagocytic Syndrome Presented as Acute Parotitis

引言：菊地氏病（Kikuchi disease）屬一種罕見的良性疾病，主要在亞裔人種觀察到；噬血症候群（hemophagocytic syndrome）則為一種少見的血液疾病，有可能導致危及生命的多重器官衰竭；在文獻報告上，嗜血症候群曾經零星地被報告出現在菊地氏症的病人身上，但鮮有合併腮腺炎的個案。病例：1名28歲的男性因雙側急性腮腺炎及持續高燒入院治療，其腮腺腫持續進行並進犯至頸部，造成呼吸道的壓迫，頸部電腦斷層檢查顯示有散漫性的唾液腺腫脹及深頸部淋巴結出現外並無膿腫形成，病人隨後接受了緊急氣管切開術；鼠蹊部淋巴結及骨髓切片檢查確立其診斷乃為菊地氏症合併噬血症候群。在接受支持性療法之後，病人順利離院，總住院天數為26天；追蹤1年後，並無疾病復發的徵象。討論：雖然臨床上菊地氏病合併噬血症候群相當罕見，在遇到非典型表現的頸部腫大時，耳鼻喉科醫師仍需謹慎將此病症納入鑑別診斷。（台耳醫誌 2012; 47:68-74）

關鍵字

Kikuchi disease ； hemophagocytic syndrome ； lymph node ； parotitis

並列摘要

INTRODUCTION: Kikuchi disease (KD), is a rare, benign, self-limiting disease reportedly primarily in Asians. Hemophagocytic syndrome (HPS) is an uncommon hematologic disorder that often results in fatal multi-organ failure. KD has been infrequently reported in association with HPS, particularly presented as acute parotitis.CASE PRESENTATION: A 28-year-old male initially presented with bilateral acute parotitis and persistent fever. Progressive parotid swelling extending into the neck region causing airway obstruction necessitated emergency tracheostomy. Final inguinal lymph node and bone marrow biopsies confirmed the diagnosis of KD accompanied with HPS. Subsequent to supportive therapy, the patient was discharged from the hospital 26 days after admission. One year later, the patient remained asymptomatic without any signs of recurrence.DISCUSSION: KD associated with HPS is rarely reported in otolaryngology. The otolaryngologist should recognize the potential for KD with HPS in atypical lesion of the head and neck. (J Taiwan Otolaryngol Head Neck Surg 2012; 47:68-74)

並列關鍵字

菊地氏病；噬血症候群；淋巴結；腮腺炎

國際替代計量

Kikuchi Disease Associated with Hemophagocytic Syndrome Presented as Acute Parotitis

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