Abstract
P 041
Extranodal marginal zone lymphoma of the lacrimal gland
Stephan Eckert, Gerhard K. Lang, Susanne Butros
Universitäts-Augenklinik Ulm
Objective
Malignant lymphomas primary developing outside the lymph nodes are characterized as extranodal lymphomas. The heterogenous lymphoproliferative lesions of the ocular adnexa vary from benign reactive lymphatic hyperplasia and “borderline lesions” to malignant lymphomas. They are rare representing 8% of all extranodal lymphoma and much more common in women.
Methods
A 52-year-old woman with swelling of her left upper lid is presented.
Results
The patient presented with a slowly progressive swelling of her left upper lid since 3 months. Optic atrophy on both eyes is known since 1979 when a craniopharyngioma has been operated. The best corrected visual acuity was 0,4 on the right eye and hand motion on the left eye because of strabismic amblyopia. Orthoptic examination showed hypotropia and reduced elevation on the left eye, Goldmann perimetry a remaining visual field nasal on the right and in the upper nasal quadrant on the left eye. Arterial hypertension was also known. An enlarged contrast-enhanced lacrimal gland was found on the left with magnetic resonance tomography. Sinusitis could be excluded by the ENT department. Because there was no change after a 2 week period of antibiotic therapy a lacrimal gland biopsy was carried out. Histology disclosed a parvicellular extranodal marginal zone lymphoma of the lacrimal gland with obvious lymphoepithelial lesions and a proliferation rate of 15-20%. In the medical department Doxycyclin therapy was started but showed no effect. Therefore a treatment with four cycles of Rituximab was begun resulting in a considerable regression.
Conclusions
The extranodal marginal zone lymphoma is the most common lymphatic neoplasia of the ocular adnexa. It is supposed that antigen stimulation but also bacterial infections can be responsible for the development of those lymphomas. An adequate biopsy is necessary for the frequently difficult classification. In addition to antibiotic therapy with for example Doxycylin, the new CD-20 antibody Rituximab can be a good alternative to the radiotherapy.
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