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Primäres intraokuläres Lymphom

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Zusammenfassung

Das primäre intraokuläre Lymphom (PIOL) ist ein seltenes extranodales Non-Hodgkin-Lymphom, das die Netzhaut, den Subretinalraum, den Glaskörper und/oder den N. opticus befallen kann. Die Erkrankung kann auf die okulären Strukturen beschränkt sein oder in Verbindung mit einem primären Lymphom des Zentralnervensystems auftreten. Der klinische Verlauf des Krankheitsbildes beginnt oft als eine steroidresistente Uveitis; dieses unspezifische Erscheinungsbild und die anspruchsvolle histopathologische Diagnostik verzögern häufig die Diagnose. Die zytologische Untersuchung von Glaskörperproben stellt derzeit den Maßstab zum Ausschluss eines PIOLs dar. Zusätzliche Techniken, insbesondere die Polymerasekettenreaktion zur Analyse klonaler Umlagerung der Immunglobulinschwerkettengene oder der T-Zell-Rezeptorgene, können weitere wertvolle Zusatzinformationen liefern. Darüber hinaus erhöhen chorioretinale Biopsien die Sicherheit der Diagnosestellung in schwierigen Fällen. Die Standardtherapie der PIOL besteht aus einer systemischen Chemotherapie. Okuläre Rezidive werden häufig mit einer perkutanen Strahlentherapie behandelt, darüber hinaus gewinnt die intraokulare Methotrexatgabe eine zunehmende Verbreitung. Auch wenn die Langzeitprognose der Patienten mit PIOL als schlecht einzustufen ist, kann in den letzten Jahren eine Verlängerung der durchschnittlichen Überlebenszeit beobachtet werden.

Abstract

Primary intraocular lymphoma (PIOL) is a rare extranodal Non-Hodgkin lymphoma (NHL), involving the retina, the subretinal space, vitreous body and/or optic nerve. The majority of PIOL are diffuse large cell B-cell lymphomas according to the new W.H.O. lymphoma classification with an immunophenotype suggesting an origin from germinal centre cells. PIOL occurs independently or together with primary central nervous system lymphoma (PCNSL), and often presents in the form of a steroid-resistent uveitis. PIOL is one of the most challenging intraocular tumours to diagnose. Cytological examination of vitreal aspirates remains the gold standard for exclusion of neoplastic disease in patients with idiopathic uveitis. Various techniques, particularly the polymerase chain reaction analysing clonal rearrangements of the immunoglobulin heavy chain or the T-cell receptor genes, prove to be useful adjuncts. Chorioretinal biopsies increase the chances of diagnosing or excluding a PIOL involving the retina and choroid. Furthermore, they allow exact subtyping of the malignant lymphoma when present, enabling exclusion of an ocular manifestation of a systemic lymphoma. Currently, most PIOL/PCNSL are treated with systemic chemotherapy. Ocular recurrences are often treated with radiotherapy, and increasingly with intraocular methotrexate. Although the prognosis of patients with PCNS/PIOL remains poor, newer methods enabling earlier diagnosis establishment and treatment are gradually increasing overall survival.

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Authors and Affiliations

  1. Institut für Pathologie, Charité Universitätsmedizin Berlin, Campus Benjamin Franklin, Berlin

    S. E. Coupland

  2. Universitäts-Augenklinik, Charité Universitätsmedizin Berlin, Campus Benjamin Franklin, Berlin

    H. Heimann

  3. Institut für Pathologie, Charité Universitätsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, 12200, Berlin

    S. E. Coupland

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  1. S. E. Coupland
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  2. H. Heimann
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Correspondence to S. E. Coupland.

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Coupland, S.E., Heimann, H. Primäres intraokuläres Lymphom. Ophthalmologe 101, 87–100 (2004). https://doi.org/10.1007/s00347-003-0855-6

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  • DOI: https://doi.org/10.1007/s00347-003-0855-6

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