Concomitant MDS with isolated 5q deletion and MGUS

• Verfasst von: Nolte, Florian [VerfasserIn]
• Verfasst von: Mossner, Maximilian [VerfasserIn]
• Verfasst von: Jann, Johann-Christoph [VerfasserIn]
• Verfasst von: Nowak, Daniel [VerfasserIn]
• Verfasst von: Boch, Tobias [VerfasserIn]
• Verfasst von: Müller, Nadine Zoé [VerfasserIn]
• Verfasst von: Hofmann, Wolf-Karsten [VerfasserIn]
• Verfasst von: Metzgeroth, Georgia [VerfasserIn]
• Titel: Concomitant MDS with isolated 5q deletion and MGUS
• Titelzusatz: case report and review of molecular aspects
• Verf.angabe: Florian Nolte, Maximilian Mossner, Johann-Christoph Jann, Daniel Nowak, Tobias Boch, Nadine Zoe Müller, Wolf-Karsten Hofmann, Georgia Metzgeroth
• Jahr: 2017
• Jahr des Originals: 2016
• Umfang: 9 S.
• Fussnoten: First published: 15 November 2016 ; Gesehen am 18.07.2018
• Titel Quelle: Enthalten in: European journal of haematology
• Ort Quelle: Oxford : Wiley-Blackwell, 1987
• Jahr Quelle: 2017
• Band/Heft Quelle: 98(2017), 3, Seite 302-310
• ISSN Quelle: 1600-0609
• DOI: doi:10.1111/ejh.12827
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: hematopoiesis and hematopathology
• Sach-SW: multiple myeloma
• Sach-SW: myelodysplastic syndromes
• Sach-SW: stem cell biology
• K10plus-PPN: 1577730798

Abstract

Patients with monoclonal gammopathy of undetermined significance (MGUS) have a higher risk for the development of concomitant primary cancers such as multiple myeloma (MM) and myelodysplastic syndrome (MDS). We report the case of patient initially suffering from MGUS of the IgG lambda subtype for more than 10 yr, which evolved to MM and MDS with deletion (5q) with severe pancytopenia. Due to pancytopenia, he received dose-reduced treatment with lenalidomide and dexamethasone. He achieved an ongoing transfusion independency after about 1 month of treatment. Bone marrow taken 14 months after start of treatment showed a complete cytogenetic response of the del(5q) clone and a plasma cell infiltration below 5%. In contrast to the development of MM in MGUS patients, the subsequent occurrence of MDS after diagnosis of MGUS is infrequent. Moreover, the biological association of MDS with MGUS is not sufficiently understood, but the non-treatment-related occurrence supports the pathogenetic role of pre-existing alterations of stem cells. Here, we summarize data on concomitant MDS and MGUS/MM with particular emphasis on molecular aspects.