Zusammenfassung
Das multiple Myelom gehört zu den hämatologischen Neoplasien und wird als B-Zell-Lymphom klassifiziert. Es entsteht aus klonalen Plasmazellen, als deren erster fassbarer Ausdruck die monoklonale Gammopathie unbestimmter Signifikanz (MGUS) zu beobachten ist. Sie erklärt sich aus der Bildung überschüssiger intakter Immunglobulinmoleküle ohne Antigenspezifität. Möglich ist auch die alleinige Bildung von Immunglobulinleichtketten. Die MGUS bedarf keiner spezifischen Therapie. Abhängig vom Progressionsrisiko erfolgt die Kontrolle der Betroffenen. Die Symptome des manifesten Myeloms sind vielfältig und häufig uncharakteristisch. Die Diagnostik umfasst die Quantifizierung des monoklonalen Proteins in Serum und Urin, die Untersuchung von Blutbild, Elektrolyten und Nierenfunktion, die Bildgebung des Skeletts und die Knochenmarkpunktion. Tragende Säule der Therapie ist das Alkylans Melphalan oder Cyclophosphamid in Kombination mit den „neuen Substanzen“ (Bortezomib, Thalidomid oder Lenalidomid).
Abstract
Multiple myeloma (MM) is a cancer originating from terminally differentiated B lymphocytes, the plasma cells and is classified as a B cell non-Hodgkin lymphoma. As clonal plasma cells secrete immunoglobulin molecules (lacking antigenic specificity), an “M component” can incidentally be detected. Besides intact immunoglobulin molecules, free light chains can be produced. Although there is no specific treatment for monoclonal gammopathy of undetermined significance (MGUS), which is the defined as the presence of clonal bone marrow plasma cells and low levels (serum and/or urine) of the M component, it should be followed up in affected individuals. The symptoms of MM are numerous and often nonspecific. Diagnosis includes the quantification of monoclonal proteins in serum and urine, blood count, electrolytes and renal function, imaging of the skeleton and bone marrow puncture. The cornerstone of therapy includes melphalan- or cyclophosphamide-based regimens incorporating one of the “novel drugs” (i.e. bortezomib, thalidomide, or lenalidomide).
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Einhaltung der ethischen Richtlinien
Interessenkonflikt. K.M. Kortüm, L. Rasche und S. Knop geben an, dass kein Interessenkonflikt besteht. M. Engelhardt: Forschungsförderung durch Janssen/Celgene/MSD; Honorare von Janssen/MSD/Mundipharma. H. Einsele: Forschungsförderung durch Janssen/Celgene; Honorare von Janssen/Celgene/Novartis. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.
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Kortüm, K., Engelhardt, M., Rasche, L. et al. Das multiple Myelom. Internist 54, 963–977 (2013). https://doi.org/10.1007/s00108-013-3336-2
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DOI: https://doi.org/10.1007/s00108-013-3336-2
Schlüsselwörter
- Monoklonale Gammopathie unklarer Signifikanz
- Knochenmarkuntersuchung
- Klassifikation
- Melphalan
- Cyclophosphamid