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„In-situ-Lymphome“ und andere Frühstadien maligner Non-Hodgkin-Lymphome

In situ lymphoma and other early stage malignant non-Hodgkin lymphomas

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Zusammenfassung

Der zunehmende Einsatz immunhistochemischer und molekularpathologischer Untersuchungen an lymphatischem Gewebe führt zu einer häufigeren Identifikation von „frühen“ Lymphoproliferationen. Diese weisen einige Merkmale maligner Lymphome auf, erfüllen jedoch klinisch und pathologisch nicht die für eine Lymphomdiagnose erforderlichen Kriterien. Neben den bereits länger bekannten prämalignen B-Zell-Neoplasien, wie der monoklonalen Gammopathie unklarer Signifikanz (MGUS) oder der monoklonalen B-Zell-Lymphozytose (MBL), wurden in den letzten Jahren In-situ-Lymphome, z. B. das follikuläre Lymphom in situ, beschrieben. Bei diesen lassen sich in morphologisch reaktivem lymphatischem Gewebe minimale, oft in typischer Lokalisation situierte Infiltrate klonaler B-Zellen darstellen, die immunphänotypisch und molekulargenetisch lymphomtypische Merkmale zeigen. Über die Frühformen von B-Zell-Neoplasien hinaus behandelt diese Übersicht einzelne Entitäten indolenter klonaler Lymphoproliferationen, wie das follikuläre Lymphom vom pädriatischen Typ und das pädiatrische nodale Marginalzonenlymphom. Dargestellt werden die diagnostischen Kriterien für „frühe“ prämaligne Lymphoproliferationen und ihre Abgrenzung gegenüber partiellen Lymphominfiltraten. Nicht berücksichtigt werden in dieser Arbeit virusinduzierte Lymphoproliferationen, die eine Grauzone zwischen rein reaktiven und neoplastischen Prozessen darstellen.

Abstract

The increasing use of immunohistochemical and molecular investigations of lymphatic tissues results in more frequent detection of early lymphoid proliferations. These show some but not all features of malignant lymphomas without fulfilling the diagnostic criteria for the diagnosis of lymphoid malignancy. In addition to well-known premalignant B-cell proliferations, such as monoclonal gammopathy of unknown significance (MGUS) and monoclonal B-cell lymphocytosis (MBL), so-called in situ lymphomas have recently been described with minimal infiltrates of clonal B-cells in morphologically reactive lymphoid tissues which show the phenotypic and genetic features of specific B-cell lymphoma subtypes and often show a characteristic topographical distribution. This article addresses a group of clonal lymphoproliferations with usually localized disease and excellent clinical prognosis, such as pediatric follicular lymphoma and nodal marginal zone lymphoma. Another group of early lesions not addressed in this review are virally induced lymphoproliferations which represent a grey zone between purely reactive lesions and malignant lymphomas and may pose significant diagnostic as well as clinical problems. In this review diagnostic criteria for early or in situ lesions and their distinction from partial infiltration by malignant lymphoma are described.

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Correspondence to L. Quintanilla-Martínez.

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Quintanilla-Martínez, L., Adam, P. & Fend, F. „In-situ-Lymphome“ und andere Frühstadien maligner Non-Hodgkin-Lymphome. Pathologe 34, 244–253 (2013). https://doi.org/10.1007/s00292-013-1748-3

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