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Erschienen in: Zeitschrift für Rheumatologie 5/2006

01.09.2006 | Übersichten

Die T-Large Granular Lymphocyte Leukämie (T-LGL-Leukämie) –

Eine wichtige Differenzialdiagnose zum Felty-Syndrom

verfasst von: Dr. med. F. Moosig, Dr. med. R. Schoch, Prof. Dr. med. Dr. rer. nat. M. Kneba

Erschienen in: Zeitschrift für Rheumatologie | Ausgabe 5/2006

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Zusammenfassung

Die T-Large Granular Lymphocyte Leukämie (T-LGL-Leukämie) ist eine seltene klonale Erkrankung, die durch Neutropenie und/oder Anämie gekennzeichnet ist. Da sie häufig mit einer Rheumatoiden Arthritis (RA) assoziiert ist, stellt sie eine wichtige Differenzialdiagnose zum Felty-Syndrom da. Da bei schwer verlaufender RA mit extraartikulären Manifestationen häufig stark expandierte Memory-Effekor-T-Zellpopulationen auftreten, die sich immunphänotypisch kaum von T-LGL-Leukämie-Zellen unterscheiden lassen, kann diese Differenzialdiagnose sehr schwierig sein. Wichtigstes Kriterium ist dann der molekulargenetische Nachweis einer monoklonalen T-Zellpopulation bei der T-LGL-Leukämie. Die Erstlinientherapie besteht in einer wöchentlichen niedrig dosierten Methotrexatgabe. Alternativ können andere Immunsuppressiva oder Zytostatika eingesetzt werden, wobei kaum Daten aus Therapiestudien vorliegen. Im Rahmen der Deutschen CLL-Studiengruppe wird eine Therapiestudie mit Erstlinien parenteraler Methotrexatgabe und Zweitlinientherapie mit Fludarabin durchgeführt.
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Metadaten
Titel
Die T-Large Granular Lymphocyte Leukämie (T-LGL-Leukämie) –
Eine wichtige Differenzialdiagnose zum Felty-Syndrom
verfasst von
Dr. med. F. Moosig
Dr. med. R. Schoch
Prof. Dr. med. Dr. rer. nat. M. Kneba
Publikationsdatum
01.09.2006
Verlag
Springer-Verlag
Erschienen in
Zeitschrift für Rheumatologie / Ausgabe 5/2006
Print ISSN: 0340-1855
Elektronische ISSN: 1435-1250
DOI
https://doi.org/10.1007/s00393-005-0013-4

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