Ocular adnexal non-Hodgkin’s lymphoma: a review of epidemiology and risk factors

Abstract

Ocular adnexal non-Hodgkin’s lymphoma (NHL), the most common form of ophthalmic NHL, has a unique incidence pattern showing a steady and rapid increase in the past few decades, nearly equal rates among both genders, and predominance among Asians/Pacific Islanders. No major cause for ocular adnexal NHL has been identified, although infectious agents, immune disorders and genetic/epigenetic factors have all been implicated in its etiology. Identifying putative risk factors and biologic mechanisms leading to carcinogenesis in ocular adnexal NHL may enable implementation of effective preventive and/or therapeutic approaches for this malignancy. This article summarizes current knowledge on epidemiology of ocular adnexal NHL and the role of various potential risk factors in its etiology.

Descriptive epidemiology of ophthalmic and ocular adnexal non-Hodgkin’s lymphoma

“…No major causative factor for ocular adnexal NHL has been identified; however, several pathogens, autoimmune processes and genetic factors have been implicated in its etiology (reviewed in [27]). There is much debate in the literature with respect to the possible causative role of pathogens in predisposition to ocular adnexal NHL (reviewed in [27]).…”

“…There is much debate in the literature with respect to the possible causative role of pathogens in predisposition to ocular adnexal NHL (reviewed in [27]). Pathogenic infections potentially relevant to the descriptive patterns of ocular adnexal NHL involve two different species of Chlamydia , namely Chlamydia trachomatis and Chlamydia psittaci.…”

Unilateral conjunctival infiltration of Adult T-cell leukemia/lymphoma. Case report and literature review

“…3 Conjunctival lymphoma is one type of ocular adnexal lymphoma. 4 Other ocular adnexal lymphomas exhibit infiltrating lymphoma cells into the eyelid and orbit. 4 Only 1-2% of all non-Hodgkin lymphoma cases are ocular adnexal lymphomas.…”

“…4 Other ocular adnexal lymphomas exhibit infiltrating lymphoma cells into the eyelid and orbit. 4 Only 1-2% of all non-Hodgkin lymphoma cases are ocular adnexal lymphomas. 5 In a previous study involving 108 cases of ocular adnexal lymphoma, infiltration into the orbit was observed in 64%, into the conjunctiva in 28%, and into the eyelid in 8%.…”

Expression Level of Valosin Containing Protein is Associated with Prognosis of Primary Orbital MALT Lymphoma

“…A substantial percentage of NHL arise from tissues other than lymph nodes and even from sites which normally contain no lymphoid tissue (Padhi et al, 2012). In recent years, the incidence of orbital and ocular adnexal MALT lymphoma is increasing worldwide (Sjö et al, 2008;Moslehi et al, 2011). According to the ILSG classification, MALT lymphoma irrespective of origin is thought to be an indolent disease as reflected by the good general prognosis of patients (Smiljanic et al, 2013).…”