Sjö Lene Dissing
Eye Pathology Institute, Department of Neuroscience and Pharmacology, Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark.
Acta Ophthalmol. 2009 Feb;87 Thesis 1:1-20. doi: 10.1111/j.1755-3768.2008.01478.x.
With a lifetime risk of 1% and 700 new cases per year, Non-Hodgkin lymphoma (NHL) is the seventh most frequent type of cancer in Denmark. The incidence of NHL has increased considerably in Western countries over the last decades; consequently, NHL is an increasing clinical problem. Ophthalmic lymphoma, (lymphoma localized in the ocular region, i.e. eyelid, conjunctiva, lacrimal sac, lacrimal gland, orbit, or intraocularly) is relatively uncommon, accounting for 5%-10% of all extranodal lymphomas. It is, however, the most common orbital malignancy. The purpose of this thesis was to review specimens from all Danish patients with a diagnosis of ophthalmic lymphoma during the period 1980-2005, in order to determine the distribution of lymphoma subtypes, and the incidence- and time trends in incidence for ophthalmic lymphoma. Furthermore, an extended analysis of the most frequent subtype, extranodal marginal zone lymphoma (MALT lymphoma), was done to analyse clinical factors and cytogenetic changes with influence on prognosis. A total of 228 Danish patients with a biopsy-reviewed verified diagnosis of ocular adnexal-, orbital-, or intraocular lymphoma were identified. We found that more than 50% of orbital- and ocular adnexal lymphomas were of the MALT lymphoma subtype, whereas diffuse large B-cell lymphoma (DLBCL) predominated intraocularly (Sjo et al. 2008a). Furthermore, lymphoma arising in the lacrimal sac was surprisingly predominantly DLBCL (Sjo et al. 2006). Incidence rates were highly dependent on patient age. There was an increase in incidence rates for the whole population from 1980 to 2005, corresponding to an annual average increase of 3.4% (Sjo et al. 2008a). MALT lymphoma arising in the ocular region was found in 116 patients (Sjo et al. 2008b). One third of patients had a relapse or progression of disease after initial therapy and relapses were frequently found at extra-ocular sites. Overall survival, however, was not significantly poorer for patients with relapse. Furthermore, we found that the frequency of translocations involving the MALT1- and IGH-gene loci is low in ocular region MALT lymphoma (2 of 42, 5%), but may predict increased risk of relapse (Sjo et al. 2008b). In conclusion the incidence of ophthalmic lymphoma is increasing at a high rate in Denmark. Ophthalmic lymphoma consists primarily of MALT lymphoma. The molecular pathogenesis of MALT lymphoma arising in the ocular region rarely involves translocations in the MALT1- and IGH-gene loci.
非霍奇金淋巴瘤（NHL）在丹麦的终生风险为1%，每年有700例新发病例，是丹麦第七大常见癌症类型。在过去几十年中，西方国家NHL的发病率显著上升；因此，NHL成为一个日益严重的临床问题。眼淋巴瘤（即局限于眼部区域，如眼睑、结膜、泪囊、泪腺、眼眶或眼内的淋巴瘤）相对少见，占所有结外淋巴瘤的5%-10%。然而，它是最常见的眼眶恶性肿瘤。本论文的目的是回顾1980年至2005年期间所有诊断为眼淋巴瘤的丹麦患者的标本，以确定淋巴瘤亚型的分布、眼淋巴瘤的发病率及发病率的时间趋势。此外，对最常见的亚型——结外边缘区淋巴瘤（MALT淋巴瘤）进行了扩展分析，以分析影响预后的临床因素和细胞遗传学变化。共确定了228例经活检复查确诊为眼附属器、眼眶或眼内淋巴瘤的丹麦患者。我们发现，超过50%的眼眶和眼附属器淋巴瘤为MALT淋巴瘤亚型，而弥漫性大B细胞淋巴瘤（DLBCL）在眼内占主导地位（Sjo等人，2008a）。此外，泪囊发生的淋巴瘤令人惊讶地主要是DLBCL（Sjo等人，2006）。发病率高度依赖于患者年龄。1980年至2005年期间整个人口的发病率有所上升，相当于年平均增长率为3.4%（Sjo等人，2008a）。在116例患者中发现了发生在眼部区域的MALT淋巴瘤（Sjo等人，2008b）。三分之一的患者在初始治疗后疾病复发或进展，且复发常发生在眼外部位。然而，复发患者的总生存率并没有显著降低。此外，我们发现眼部区域MALT淋巴瘤中涉及MALT1和IGH基因位点易位的频率较低（42例中有2例，5%），但可能预示复发风险增加（Sjo等人，2008b）。总之，丹麦眼淋巴瘤的发病率正在高速上升。眼淋巴瘤主要由MALT淋巴瘤组成。眼部区域发生的MALT淋巴瘤的分子发病机制很少涉及MALT1和IGH基因位点的易位。
