Mencía-Gutiérrez Enrique, Gutiérrez-Díaz Esperanza, Salamanca Javier, Martínez-González Miguel A
Department of Ophthalmology, 12 de Octubre Hospital, Complutense University, Madrid, Spain.
Int J Dermatol. 2006 Jun;45(6):766-9. doi: 10.1111/j.1365-4632.2004.02412.x.
To report an unusual case of cutaneous presentation on the eyelid of systemic (or nodal), CD30+, anaplastic large-cell lymphoma (ALCL).
A 39-year-old man presented with a rapidly growing exophytic mass on the left upper eyelid, with a protuberant, ulcerated aspect and with discharge. The patient showed lymph node involvement 3 months after the appearance of the lesion on the eyelid (the lesion itself appeared 1 week before examination).
The histopathologic and immunohistochemical diagnosis was ALCL, T-cell phenotype, strongly positive for CD43 and CD30, and negative for CD3, anaplastic lymphoma kinase (ALK), and B-cell antigens. Treatment was by radiotherapy and, later, chemotherapy (cyclophosphamide, adriamycin, vincristine, and prednisolone, CHOP) for skin recurrences and lymphadenopathies over 5 years. There has been no recurrence for more than 6 years.
Primary, systemic, CD30+, ALK-negative, ALCL presentations generally have a poor prognosis and tend to occur in older individuals, although the clinical outcome is highly variable and difficult to predict in individual cases. Only three cases of ALCL have been described in the ocular adnexae and none was ALK-negative.
报告一例系统性（或淋巴结）CD30+间变性大细胞淋巴瘤（ALCL）累及眼睑皮肤的罕见病例。
一名39岁男性患者，左上眼睑出现一个迅速生长的外生性肿物，肿物突出、溃疡，并有分泌物。在眼睑肿物出现3个月后患者出现淋巴结受累（肿物本身在检查前1周出现）。
组织病理学和免疫组化诊断为ALCL，T细胞表型，CD43和CD30强阳性，CD3、间变性淋巴瘤激酶（ALK）和B细胞抗原阴性。治疗采用放疗，随后针对皮肤复发和淋巴结病变进行化疗（环磷酰胺、阿霉素、长春新碱和泼尼松龙，CHOP方案），持续5年。6年多来未再复发。
原发性系统性CD30+、ALK阴性的ALCL通常预后较差，且倾向于发生在老年个体中，尽管临床结果差异很大，个别病例难以预测。眼部附属器仅报道过3例ALCL，且均非ALK阴性。
