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Systemic Mantle Cell Lymphoma with Initial Presentation of Bilateral Ptosis-A Casr Report

以雙側眼瞼下垂為初表徵之全身性淋巴瘤案例報告

摘要


目的:以雙側眼瞼下垂為初表徵之全身性淋巴瘤案例報告。方法:病例報告。結果:一名56歲女性病患,因為左側眼瞼下垂至本院求診。經檢查後可見,雙眼之臉緣反射距離(margin reflex distance)分別為右眼2.5mm,左眼0.5mm。雙側上眼瞼皆可觸診到腫塊,且以左側更為明顯。電腦斷層則顯示雙側上眼瞼皆有半月型之組織腫大情形。病人接受左側腫瘤切除後,眼瞼下垂獲得改善。腫瘤病理切片診斷為被套細胞淋巴瘤(Mantle cell lymphoma, MCL)。全身性檢查則有骨髓,縱膈腔,及上腹部淋巴結之侵犯。病人當時拒絕接受進一步化學治療及追蹤。兩年後,病人再度因為左側眼瞼下垂回診,並要求手街改善下垂情形。右側眼瞼腫瘤切除後,病理切片診斷則和左側相同。之後,她接受了支持性化學治療,並於兩年後死亡。結論:眼窩部的被套細胞淋巴瘤(Mantle cell lymphoma, MCL)相當少見,而以男性居多。過去的案例報告顯示該區域的被套細胞淋巴瘤常合併全身性的系統侵犯。往往於診斷之初,便屬於第四期疾病。我們的案例以雙側眼瞼下垂作為淋巴瘤之初始病徵,因此,進一步完整的全身性系統檢查,包括骨髓切片,對於疾病的分期及預後相當重要。而該病患雖然延遲了化學治療,然存活時間卻沒有比過去報告的案例來得短。

關鍵字

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並列摘要


Purpose: To report a rare case of systemic Mantle cell lymphoma with initial presentation of bilateral ptosis.Method: Interventional case report.Results: A 56-year-old female visited our hospital due to progressive left lid ptosis in the past three months. She had previously received four lid blepharoplasties. Soft palpable masses were noted over bilateral upper lids during examination. The margin reflex distance (MRD) of the right eye and the left eye was 2.5 mm and 0.5 mm, respectively. Orbital CT scan revealed crescent-shaped tissue swelling of bilateral upper lids. Excision of left eyelid tumor was performed, and the pathology revealed Mantle cell lymphoma of blastic variant type. Systemic survey showed bone marrow, mediastinum, and upper abdomen involvement. Further systemic chemotherapy was suggested immediately, but the patient refused any treatment at that time. After loss of follow-up for 2 years, she visited our clinic for surgical correction of her progressive right lid ptosis. After operation, she underwent palliative chemotherapy. However, the patient died 2 years later.Conclusion: There is a high proportion of systemic involvement in Mantle cell lymphoma of the eyelid. Our patient presented with systemic disease and rapidly progressive ptosis. Despite of delayed chemotherapy, the survival in our case is comparable with those of other reports.

並列關鍵字

Mantle cell lymphoma Eyelid Ptosis

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