Zusammenfassung
Das splenische Marginalzonen-B-Zell-Lymphom (SMZBL) ist eine seltene, organotypische Lymphomerkrankung, die einzigartige klinische und pathologische Eigenschaften aufweist. Bei der initialen Manifestation der Erkrankung sind üblicherweise die Milz, das Knochenmark und häufig auch das periphere Blut betroffen. Eine generalisierte Lymphadenopathie hingegen ist selten. Die molekularen Charakteristika der Erkrankung sind der Nachweis einer somatischen Hypermutation der IgVH-Gene in etwa der Hälfte der Fälle und der häufige Nachweis (45%) heterozygoter Deletionen im langen Arm des Chromosoms 7. Etwa 10–15% der Fälle zeigen eine Assoziation zu einer chronischen Hepatitis-C-Virus- (HCV-)Infektion, ein Befund, der zumindest in diesen Fällen, wahrscheinlich aber auch in den nicht-HCV-assoziierten Fällen des SMZBL eine antigengetriggerte Proliferation der Tumorzellen nahe legt. Jüngere Daten, die ein homogenes Genexpressionsmuster des SMZBL zeigen konnten, belegen, dass das SMZBL eine distinkte Tumorentität darstellt. Der klinische Verlauf ist überwiegend indolent, ein Teil der Patienten weist jedoch einen aggressiveren Verlauf auf, der mit bestimmten molekularen Charakteristika des Tumors (unmutierter IgVH-Status und 7q-Deletionen) assoziiert ist.
Abstract
Splenic marginal zone B cell lymphomas (SMZBCL) are rare, organotypic, lymphoid neoplasms with distinct clinicopathological features. At initial presentation, the spleen, bone marrow and peripheral blood are usually involved, while generalized lymphadenopathy is only rarely observed. Molecularly, somatic hypermutation of IgVH genes can be detected in roughly half of the cases, and deletions in 7q are present in 45% of tumors. Approximately 10%–15% of SMZBCL do occur in the setting of chronic hepatitis C. This association underlines the importance of antigenic stimulation in the proliferation of the tumor cells in HCV-associated SMBCL, if not also in their classical counterparts. More recently, gene profiling studies using cDNA microarrays revealed a homogeneous expression profile in SMZBCL, thus further confirming the notion of a distinct tumor entity. The clinical course is indolent in the majority of cases; however, some patients follow a more aggressive clinical course, usually associated with some particular molecular features in these tumors, such as unmutated IgVH genes and 7q deletions.
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Ott, M., Müller-Hermelink, H. Splenische Marginalzonen-B-Zell-Lymphome. Pathologe 29, 143–147 (2008). https://doi.org/10.1007/s00292-008-0969-3
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DOI: https://doi.org/10.1007/s00292-008-0969-3