Zusammenfassung
Die früher als Retikulumzellsarkome bezeichneten, hochmalignen Non-Hodgkin Lymphome befallen in ihrer okulozerebralen Form Auge und Zentralnervensystem älterer Patienten. Immunoblastische Lymphome sind selten, meist sind beide Augen, wenn auch in unterschiedlichem Zeitabstand und Ausmaß betroffen. Der okuläre Befall geht der Generalisierung der Erkrankung in Form der ZNS-Beteiligung um Monate bis Jahre voraus. Wir berichten über einen 62jährigen Patienten, der von Sept. 88 bis Sept. 89 bei unauffälligem internen Status eine langsam progrediente Panuveitis des rechten Auges entwickelte. Über Monate bildeten sich disseminierte Pigmentepithelabhebungen, zunehmende Glaskörpertrübungen, ein solides Pseudohypopyon mit schlammartiger Ansammlung von Tumorzellen in der Vorderkammer und ein Sekundärglaukom. Im Nov. 89 kam es zu einer massiven Papillenschwellung links, die Durchführung einer diagnostischen Vitrektomie wurde jedoch vom Patienten zu wiederholten Malen abgelehnt. Die immunhistochemische Aufarbeitung des enukleierten rechten Bulbus ergab ein hochmalignes, polymorphes, zentroblastisches Lymphom mit vielen Immunoblasten. Der Patient unterzog sich einer Radiatio des Neurocraniums und des Spinalkanales, eine intrathekale und intravenöse Chemotherapie wurde durchgeführt. Innerhalb der Nachbeobachtungszeit von über 2 Jahren traten keinerlei okuläre oder zerebrale Lymphom-Rezidive auf, am linken Auge konnte bei klar gebliebener Linse der volle Visus erhalten werden.
Summary
A rare form of malignant large-cell non-Hodgkin’s lymphoma or reticulum cell sarkoma is confined to the eye and central nervous system (CNS). We report a 62-year old man, who presented with recalcitrant progressive uveitis in his right eye. Although lymphoma was suspected, the patient refused to undergo vitrectomy. Over one year he developed large blister-like solid scattered lesions of the retinal pigment epithelium (RPE) in the same eye. Repetitive Neuroimaging could not detect any CNS involvement. After developing optic disc-swelling and discrete RPE-changes in the left eye also, the patient underwent enucleation of his right eye. Histopathology and Immunohistochemistry revealed malignant intraocular large cell lymphoma, invading the whole eye. Liquor cytology showed lymphomatous cells. Suffering from high grade malignant lymphoma with CNS involvement, the patient had to undergo radiation therapy of the orbits and neuroachsis as well as intravenous and intrathecal chemotherapy. Papilledema of the left eye resolved quickly and completely, RPE-infiltrations disappeared and led to discrete punched out lesions with no scarring, the lens remained transparent over the whole observation period of 25 months. The patient is surviving for more than 52 months after first suspicion of malignant lymphoma, he has retained full visual acuity in his left eye until now.
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Arocker-Mettinger, E., Huber-Spitzy, V., Stur, M. et al. Cortisonresistente Panuveitis — entlarvt als Retinales Lymphom. Spektrum Augeheilkd 7, 90–96 (1993). https://doi.org/10.1007/BF03163986
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DOI: https://doi.org/10.1007/BF03163986
Schlüsselwörter
- Cortisonresistente panuveitis
- polymorphes
- zentroblastisches lymphom
- intrathekale und intravenöse Chemotherapie
- Radiatio des Neurocraniums