van de Donk, Niels W C J5; Palumbo, Antonio6; Johnsen, Hans Erik7; Engelhardt, Monika8; Gay, Francesca6; Gregersen, Henrik1; Hajek, Roman9; Kleber, Martina8; Ludwig, Heinz10; Morgan, Gareth22; Musto, Pellegrino12; Plesner, Torben13; Sezer, Orhan14; Terpos, Evangelos15; Waage, Anders16; Zweegman, Sonja17; Einsele, Hermann18; Sonneveld, Pieter19; Lokhorst, Henk M20
1 The Faculty of Medicine, Aalborg University, VBN2 Aalborg University Hospital, The Faculty of Medicine, Aalborg University, VBN3 Blodsygdomme (Hæmatologi), The Faculty of Medicine, Aalborg University, VBN4 Klinik Medicin, The Faculty of Medicine, Aalborg University, VBN5 Department of Hematology, University Medical Center Utrecht, The Netherlands firstname.lastname@example.org Divisione di Ematologia dell'Università di Torino, Azienda Ospedaliero-Universitaria Città della Salute e della Scienza, Turin, Italy.7 Department of Clinical Medicine, The Faculty of Medicine, Aalborg University, VBN8 Department of Hematology and Oncology, University of Freiburg Medical Center, Germany.9 Department of Haemato-oncology, University Hospital Ostrava and Faculty of Medicine, Czech Republic.10 Department of Medicine I, Wilhelminenspital Wien, Austria.11 Royal Marsden Hospital London12 Scientific Direction, Centro di Riferimento Oncologico della Basilicata, Istituto di Ricovero e Cura a Carattere Scientifico, Rionero in Vulture, Italy.13 Department of hematology at Vejle Hospital, Denmark.14 Department of Hematology, Memorial Hospital, Istanbul, Turkey.15 Department of Clinical Therapeutics, National and Kapodistrian University of Athens School of Medicine, Greece.16 Department of Hematology, St. Olavs Hospital, Norwegian University of Science and Technology, Trondheim, Norway.17 Department of Hematology, VU University Medical Center, Amsterdam, The Netherlands.18 Universitätsklinik Würzburg, Medizinische Klinik und Poliklinik II, Würzburg, Germany.19 Department of Hematology, Erasmus Medical Center, Rotterdam, The Netherlands.20 Department of Hematology, University Medical Center Utrecht, The Netherlands.21 unknown22 Royal Marsden Hospital London
recommendations from the European Myeloma Network
Monoclonal gammopathy of undetermined significance is one of the most common pre-malignant disorders. IgG and IgA monoclonal gammopathy of undetermined significance are precursor conditions of multiple myeloma; light-chain monoclonal gammopathy of undetermined significance of light-chain multiple myeloma; and IgM monoclonal gammopathy of undetermined significance of Waldenström's macroglobulinemia and other lymphoproliferative disorders. Clonal burden, as determined by bone marrow plasma cell percentage or M-protein level, as well as biological characteristics, including heavy chain isotype and light chain production, are helpful in predicting risk of progression of monoclonal gammopathy of undetermined significance to symptomatic disease. Furthermore, alterations in the bone marrow microenvironment of monoclonal gammopathy of undetermined significance patients result in an increased risk of venous and arterial thrombosis, infections, osteoporosis, and bone fractures. In addition, the small clone may occasionally be responsible for severe organ damage through the production of a monoclonal protein that has autoantibody activity or deposits in tissues. These disorders are rare and often require therapy directed at eradication of the underlying plasma cell or lymphoplasmacytic clone. In this review, we provide an overview of the clinical relevance of monoclonal gammopathy of undetermined significance. We also give general recommendations of how to diagnose and manage patients with monoclonal gammopathy of undetermined significance.