Zusammenfassung
Plasmazellneoplasien sind Tumoren terminal differenzierter B-Zellen, in denen Plasmazellen den dominanten und proliferierenden Tumorzellanteil darstellen. Das Plasmazellmyelom (PCM) ist eine der häufigsten hämatologischen Neoplasien und bereitet in typischen Fällen keine diagnostischen Schwierigkeiten. Eine morphologisch und immunphänotypisch nachweisbare plasmazelluläre oder plasmoblastische Differenzierung kann jedoch bei einer Vielzahl reifer B-Zell-Lymphome auftreten und im Einzelfall differenzialdiagnostische Probleme aufwerfen. Eine sichere Abgrenzung der verschiedenen Entitäten erfordert die Integration klinischer und morphologischer Befunde sowie eine adäquate Phänotypisierung sowohl der Plasmazell- als auch, wenn vorhanden, der B-Zell-Komponente. Der Nachweis lymphotroper Viren, spezifischer Translokationen sowie neuer molekularer Marker, wie der MYD88-L265P-Mutation, in der großen Mehrheit der lymphoplasmozytischen Lymphome ergänzen das diagnostische Repertoire. In dieser Übersicht werden die in der diagnostischen Praxis am häufigsten beobachteten Abgrenzungsschwierigkeiten zwischen niedrigmalignen B-Zell-Non-Hodgkin-Lymphomen (B-NHL) mit plasmazellulärer Komponente und dem PCM einerseits und zwischen hochmalignen B-NHL, insbesondere dem plasmablastischen Lymphom, und dem aggressiven PCM mit extramedullärer Ausbreitung andererseits dargestellt. Damit soll eine Hilfestellung für die praktische Diagnostik gegeben werden.
Abstract
Plasma cell malignancies are tumors of terminally differentiated B-cells in which the neoplastic plasma cells are the dominant and proliferating tumor cell component. Plasma cell myeloma (PCM) is one of the most common hematological neoplasms and typically does not cause diagnostic problems. A morphologically and immunophenotypically detectable plasmacellular orplasmablastic differentiation is, however, commonly observed in a wide range of mature B-cell lymphomas. A confident separation of the distinct entities requires the integration of clinical and morphological findings as well as an adequate phenotyping of both the plasma cell and the B-cell component if present. Detection of lymphotropic viruses, specific translocations and novel molecular markers, such as the MYD88 L265P mutation occurring in the vast majority of lymphoplasmacytic lymphomas complement our diagnostic repertoire. In this review we describe the most commonly observed diagnostic problems in separating small B-cell lymphomas from PCM and high-grade B-cell non-Hodgkin lymphoma (B-NHL) with plasmablastic differentiation from extramedullary spread of aggressive PCM and provide helpful criteria for routine diagnostics.
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Fend, F., Quintanilla-Martínez, L. B-Zell-Neoplasien mit plasmazellulärer und plasmablastischer Differenzierung. Pathologe 34, 198–209 (2013). https://doi.org/10.1007/s00292-013-1743-8
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DOI: https://doi.org/10.1007/s00292-013-1743-8