Abstract

The aetiology of OAL is undefined, although much attention has been recently focused on determining whether OAL is caused by an autoimmune disorder, chronic antigenic stimulation or both. It is becoming evident that infectious agents underlying chronic eye infection, as Chlamydia, may play a role in ocular lymphomagenesis. The high prevalence of Chlamydophila psittaci in patients with OAL has suggested a potential oncogenic role for its tendency to cause chronic and persistent infections, although it has been documented an evident geographical variability and response to antibiotic treatment. For C. pneumoniae, the findings so far obtained are very limited not only for identification in OAL but also for the specific treatment with antibiotics. The recent molecular and cultural evidence of C. trachomatis in patients with OAL, seems to suggest that also this pathogen may contribute to pathogenesis of such lymphoma. The potential application of bacteria-eradicating therapy at local and systemic level may ultimately result in safer and more efficient therapeutic option for patients affected by these malignancies. Moreover, a close collaboration between experts in ophthalmology, infectious diseases and hematology will help, in the future, to effectively manage this disease. This review attempts to weigh the currently available evidence regarding the role that Chlamydia play in development of OAL and focuses on patients with OAL observed at our Institution.

Highlights

  • Non-Hodgkin’s lymphomas represent the most common ocular adnexa neoplasms and approximately account for 5% - 15% of all extranodalmarginal zone (MZ) lymphomas, localizing at the conjunctiva, orbital soft tissue and lachrymal apparatus of the ocular adnexa [1,2]

  • The potential application of bacteria-eradicating therapy at local and systemic level may result in safer and more efficient therapeutic option for patients affected by these malignancies

  • The regression of lymphoma after antibiotic administration was often observed in patients not harboring C. psittaci DNA, suggesting that other undiscovered doxycycline-sensitive organisms may be involved in the pathogenesis of disease [110]

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Summary

Introduction

Non-Hodgkin’s lymphomas represent the most common ocular adnexa neoplasms and approximately account for 5% - 15% of all extranodalmarginal zone (MZ) lymphomas, localizing at the conjunctiva, orbital soft tissue and lachrymal apparatus of the ocular adnexa [1,2]. OALs are mostly seen in the fifth to seventh decade of life [median age, 65 years], with a female predominance and the majority (85% - 90%) of patients present with localized disease (Stage I) They can infiltrate every orbital and ocular adnexal tissue [19]. The chronic antigenic stimulation hypothesis claims that a specific infectious agent initiates a reactive lymphoid infiltrate in the normally sterile ocular adnexal tissues and leads to a B-cell clonal expansion and proliferation. At this stage, genetic alterations and microenvironment may sustain an independent growth of the infectious agent. The large number of papers reported in the literature concern with C. psittaci, there is evidence that other Chlamydiae such as C. trachomatis and C. pneumoniae can somehow be implicated in the pathogenesis of OAL

Chlamydia Morphology and Function
Pathogenesis of Chlamydia Persistence and Chronic Infection
Chlamydia psittaci
Focus on OAL Cases Admitted at Our Institution
Therapeutic Strategies in OAL
Findings
Discussion
10. Conclusions
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