Conjunctival Lymphoma

Abstract

Lymphoma of the conjunctiva is an ocular malignancy derived from clonal proliferation of lymphocytes. The majority of conjunctival lymphoma is extranodal marginal zone B-Cell lymphoma (EMZL), however diffuse large B-cell (DLBCL), follicular (FL), mantle cell (MCL) and T- cell subtypes are also seen. Clinical manifestations are non-specific, but include unilateral or bilateral painless salmon-pink conjunctival lesions. Approaches to treatment have centered around local immunomodulation, often with Interferon-α2b or Rituximab (anti-CD20 monoclonal antibody) with or without radiation. Although conjunctival lymphoma is generally considered an indolent disease, recent advances in next-generation sequencing have improved clinicians’ ability to predict future recurrence or systemic disease through assessment of cytogenic and molecular features. In this paper, we review the classification, clinical features, diagnostic techniques, and emerging strategies for management and prognostication of conjunctival lymphomas.

Translated abstract

结膜淋巴瘤是一种源于淋巴细胞克隆性增殖的眼部恶性肿瘤。大多数的结膜淋巴瘤来源于淋巴结边缘区B淋巴细胞 (EMZL), 但也可见弥漫性大B细胞 (DLBCL)、滤泡细胞 (FL)、套细胞 (MCL)以及T细胞亚型。结膜淋巴瘤的临床表现无特异性, 但包括单侧或双侧无痛性浅粉色鱼肉样结膜病变。治疗方法通常以局部免疫调节治疗为主, 通常使用干扰素-α2β或利妥昔单抗 (抗CD20单克隆抗体)伴或不伴放疗。尽管结膜淋巴瘤通常被认为是一种惰性疾病, 但新一代测序的最新进展提高了医生通过细胞遗传学和分子特征预测未来复发率及全身疾病的能力。在本文中, 我们回顾了结膜淋巴瘤的分类、临床特征、诊断技术、治疗及预后的新兴策略。