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Das mediastinale (thymische) großzellige B-Zell-Lymphom

Mediastinal large B-cell lymphoma

Zusammenfassung

Das mediastinale B-Zell-Lymphom (MBL) ist ein hochmaligner, lokal aggressiver Tumor der erstmals in den frühen 1980er Jahren beschrieben wurde. Das Lymphom hat bei einer Inzidenz von 2–3% unter den Non-Hodgkin-Lymhomen ein breites morphologisches Spektrum, was die initiale Charakterisierung dieses Lymphoms erschwerte. Da sich das MBL jedoch klinisch, morphologisch, immunologisch und zytogenetisch klar von anderen Lymphomtypen abgrenzen lässt, wurde es erstmals 1994 in der revidierten Europäisch-Amerikanischen Klassifikation lymphoider Neoplasien (REAL-Klassifikation) als eigene Entität unter den diffusen großzelligen B-Zell-Lymphomen anerkannt. In der aktuellen Klassifikation der Weltgesundheitsorganisation (WHO) der Tumoren des hämatopoetischen und lymphatischen Gewebes wird das primär mediastinale B-Zell-Lymphom mit einem eigenen Code (ICD-9679/3) gelistet.

Abstract

Mediastinal B-cell lymphoma is a locally highly aggressive tumour which was first described in the early 1980s. The incidence is about 2–3% of all non-Hodgkin’s lymphomas. The conceptional evolution of this lymphoma entity was hampered by its low incidence and the broad spectrum of morphological variants present. However, since mediastinal B-cell lymphoma has distinct morphological, immunological, genetic, and clinical features, it has been listed in the revised European-American Classification of Lymphoid Neoplasms (REAL-Classification) since 1994 as a variant of diffuse large B-cell lymphoma. In the World Health Organization classification of malignant lymphomas, mediastinal B-cell lymphoma is now listed with an own disease code (ICD-9679/3).

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Barth, T., Möller, P. Das mediastinale (thymische) großzellige B-Zell-Lymphom. Pathologe 28, 36–40 (2007). https://doi.org/10.1007/s00292-006-0880-8

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