Szemészet, 2019 (156. évfolyam, 1-4. szám)

2019-05-01 / Suplementum I.

Kongresszusi összefoglalók

A bizonytalan jelentőségű monoklonális gammopathia (MGUS), a soliter csontplazmocytoma, a Waldenström-macroglobulinaemia, az aszimptomatikus és szimptomatikus myeloma multiplex mind a plazmasejtbetegségek különféle stádiumaiba tartoznak, amelyeket monoklonális gammopathia jellemez. Szaruhártya homályok leírására monoklonális gammopathiában mintegy 70 beteg esetében került sor az irodalomban. A szaruhártya homályok nummularis vagy kristályszerűek, szürkés, vagy fehéres, esetenként vonalas lerakódásként jelennek meg a cornea stromában. Lymphoproliferatív betegség esetén e mellett uveitist, maculopathiát és centrális artériás vagy vénás okklúziót lehet esetenként a betegeknél megfigyelni.

Kurzusunk összefoglalja a monoklonális gammopathiával járó lymphoproliferatív betegségeket, a különféle szervekben esetenként bekövetkező immunglobulin depozíciót, a paraproteinémiás keratopathia klinikai jeleit és a monoklonális gammopathia szemészeti jeleinek jelentőségét.

Course 10

KID Ocular signs of monoclonal gammopathy Moderator: Nóra Szentmáry

Gábor Mikala (South-Pest Center Hospital - National Hematological and Infectological Institute, Department of Hematology and Stemcell- Transplantation, Budapest, Hungary)

Walter Lisch (Klinik für Augenheilkunde, Johannes Gutenberg Universität Mainz, Deutschland)

Nóra Szentmáry (Semmelweis University Department of Ophthalmology, Budapest, Hungary and Klinik für Augenheilkunde, Universität des Saarlandes, Homburg/Saar, Deutschland)

Monoclonal gammopathy of undetermined significance (MGUS), solitary bone plasmocytoma, Waldenstrom's disease (macroglobulinaema) and asymptomatic or symptomatic multiple myeloma are all different stages of the same disease, all with monoclonal gammopathy.

Corneal opacifications (paraproteinemic keratopathy) in monoclonal gammopathy have been described in about 70 patients patients in the literature. These may be nummular or crystal-like, or even present with white or grey line-forming depositions in the stroma. Patients with lymphoproliferative disease may also present with uveitis, maculopathy, central retinal artery or vein occlusion.

Our course summarizes lymphoproliferative diseases with monoclonal gammopathy, possible immunglobulin deposition in different organs, clinical signs of paraproteinemic keratopathy and significance of ocular signs of monoclonal gammopathy.

ESS Honorary Member Lecture

Üléselnök/Chair: Miklós Resch, Gábor Németh

The anatomy of invisible - in-vivo-confocal laser scanning microscopy of the cornea Rudolf Guthoff University of Rostock

For in-vivo examination of semitransparent tissues like the cornea slit lamp biomicroscopy is the standard technique since over a century. Optically cut planes in this technique are orientated sagitally and observed by a binocular microscope with the magnification up to 50 fold. With this magnification single cell resolution is impossible. Nevertheless, the majority of corneal diseases can be diagnosed that way and treatment follow up was easily possible in most of the patients.

Confocal in-vivo-microscopy images deal with the same unprocessed biological structures but:

1. with a magnification of approximately 800 fold which clearly allows single cell evaluation and

2. the optical section directed parallel to the corneal surface which means perpendicular to the slit lamp image.

To illustrate the clinical benefit of in-vivo CCM three examples will be given:

1. Diagnoses of keratomycosis.

In-vivo CCM has proven to be an excellent way for instant diagnoses avoiding long lasting preparations of sample cultures. Differential diagnoses of nonfungal keratitis with activation of keratocytes and instantly proven fungi in a corneal ulcer will be demonstrated.

2. The ophthalmological evaluation of the subbasal corneal nerve plexus.

In-vivo CCM with its surface parallel optical slicing of the cornea offers an ideal physical prerequisite to display and to quantify structures of the subbasal nerve plexus in a well-defined anatomical plain. This plexus is located between Bowman's membrane and the basal lamina of the corneal epithelial cells. Examples of normal and rarefied nerve fibers in this layer will be given. With this information it is hoped that new treatment strategies can be worked out for a more effective prevention of this serious disorder to and in small fiber neuropathy reduce diabetes induced complications.

3. Corneal keratocytes - a neglected entity of cells.

Keratocytes are specialized fibroblasts representing about 10% of the volume of the corneal stroma. They are described by Vogt as „corpusculi cornea" in his fundamental 3 volume textbook on slit lamp biomicroscopy in 1930. In-vivo CCM easily displays keratocytes nuclei with a cell diameter of approximately 10 pm. The star-shaped cytoplasm of keratocytes with an overall diameter of up to a 100 pm is invisible under normal circumstances.

Various noxes such as local mechanical ones by epithelial erosions or toxic ones from free oxygen radicals in corneal x-linking change the optical properties of the cytoplasm of keratocytes dramatically and makes it visible in all its complexity. There is evidence that these morphological changes appear in the process of cell death when apoptotic vesicles in the keratocyte stroma turn transparent cellular parts into an accumulation of highly scattering elements. These ghost cells finally disappear totally and after weeks are replaced by spindle shaped migrating keratocyte subtypes migrating from adjacent corneal areas.

In the last 20 years laser scanning confocal in-vivo-microscopy has found its place in clinical research, recently also heading for new information in ocular surface diseases.

65';