Abstract
Diffuse large B-cell lymphoma (DLBCL) and mantle cell lymphoma (MCL) constitute two distinct subtypes of non-Hodgkin lymphoma (NHL) displaying marked diversities with regard to molecular biology and clinical features. Because these differences could be related to the location of the lymphoma, it is of interest to investigate the clinical and histopathological features of DLBCL and MCL involving the ocular adnexal region (i.e. the orbit, eyelids, conjunctiva, lacrimal gland and lacrimal sac). Similarly, the lacrimal gland is the only glandular structure within the orbit. Because the lacrimal gland represents an important part of the immunological system, it is of interest to investigate lymphomas involving this location with regard to clinical and histological characteristics. The purpose of this thesis was to characterize the clinical and histopathological features of Danish patients with DLBCL of the ocular adnexal region between 1980 and 2009 and of Danish patients with ocular adnexal MCL from 1980 to 2005 (Rasmussen 2013). Furthermore, the aim was to review all specimens from patients with lacrimal gland lymphoma in Denmark between 1975 and 2009 to determine the distribution of lymphoma subtypes of the lacrimal gland and to describe the clinicopathological features of these patients. A total of 34 patients with DLBCL and 21 with MCL of the ocular adnexal region were identified (Rasmussen et al. 2009, 2013). Twenty-seven patients had lacrimal gland lymphoma (Rasmussen et al. 2011). Elderly patients predominated in all three groups, with median ages of 78, 75 and 69 years in the DLBCL, the MCL and the lacrimal gland lymphoma groups, respectively. Patients with MCL had a preponderance of men (Rasmussen et al. 2009), whereas women prevailed among patients with lacrimal gland lymphoma (Rasmussen et al. 2011). The orbit was the most common site of involvement in DLBCL and MCL. The majority of lacrimal gland lymphomas were low grade, and the distribution of subtypes was as follows: extranodal marginal zone lymphoma, 10 (37%); follicular lymphoma, 5 (19%); DLBCL, 4 (15%); MCL, 3 (11%); chronic lymphocytic leukaemia/small lymphatic lymphoma, 2 (7%); and unclassified B-cell lymphoma, 3 (11%) (Rasmussen et al. 2011). The 5-year overall survival rate for the DLBCL and MCL groups was 20% and 22%, respectively (Rasmussen et al. 2009; Rasmussen 2013). However, 83% of the patients with MCL who received rituximab-containing chemotherapy were alive after 5 years from the time of diagnosis. The 5-year overall survival rate of patients with lacrimal gland lymphoma was 70% (Rasmussen et al. 2011). In conclusion, DLBCL and MCL involving the ocular adnexal region, and lymphoma of the lacrimal gland are prevalent among elderly patients. The overall prognosis in DLBCL and MCL was poor, whereas the prognosis for patients with lacrimal gland lymphoma was relatively good. Chemotherapy containing rituximab significantly improved survival in patients with MCL. Note from the Editor: This PhD thesis is published as an electronic supplement, see: Rasmussen PK (2013): Diffuse large B-cell lymphoma and mantle cell lymphoma of the ocular adnexal region, and lymphoma of the lacrimal gland: an investigation of clinical and histopathological features. Acta Ophthalmol. 91 (thesis 5): 1–27
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