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Evolving Concepts in the Diagnosis and Staging of Multiple Myeloma

Presented by: Jens Hillengass

Until recently, treatment for multiple myeloma was only initiated in symptomatic disease. Greater understanding of the underlying biology and the development of new biomarkers, however, has led to changes in diagnosis, and with the help of improved imaging techniques, clinicians are increasingly identifying patients with early myeloma who may benefit from treatment. One major development in diagnostic imaging is the replacement of radiography with CT. PET/CT imaging is also useful for treatment response assessment, because the disappearance of focal lesions is an important prognostic factor. Finally, the Revised International Staging System for multiple myeloma has incorporated additional prognostic markers of aggressive disease, and risk-adapted treatment has been shown to improve survival.

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Optimizing Thromboembolism Prophylaxis for the Contemporary Age of Multiple Myeloma

Muhamed Baljevic, Douglas W. Sborov, Ming Y. Lim, Jens Hillengass, Thomas Martin, Jorge J. Castillo, Michael B. Streiff, Shaji K. Kumar, and Natalie S. Callander

Venous thromboembolism (VTE) is a major complication in all patients with cancer. Compared with the general population, patients with multiple myeloma (MM) have a 9-fold increase in VTE risk, likely because of their malignancy, its treatments, and other additional patient-related factors. In MM, thromboembolism events tend to occur within 6 months of treatment initiation, regardless of treatment regimen; however, the use of immunomodulatory agents such as thalidomide or lenalidomide, especially in combination with dexamethasone or multiagent chemotherapy, is known to create a significant risk for VTE. Currently, official recommendations for VTE prophylaxis in MM outlined in various national guidelines or multidisciplinary society panels are based on expert opinion, because data from randomized controlled trials are scarce. Large studies which have mainly focused on the efficacy of thromboprophylaxis in patients with cancer at higher risk for VTE either had a very low representation of patients with MM, or excluded them all together, limiting our ability to draw evidence-based conclusions on how to effectively protect MM population from VTE. In this brief perspective, we highlight some of the greatest challenges that have hampered the field concerning the availability of high-quality clinical trial data for the formulation of best VTE prophylaxis strategies in patients with newly diagnosed MM, as well as the rationale for the latest updates in the NCCN Guidelines on this topic.

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NCCN Guidelines® Insights: Multiple Myeloma, Version 3.2022

Featured Updates to the NCCN Guidelines

Natalie S. Callander, Muhamed Baljevic, Kehinde Adekola, Larry D. Anderson Jr, Erica Campagnaro, Jorge J. Castillo, Caitlin Costello, Srinivas Devarakonda, Noura Elsedawy, Matthew Faiman, Alfred Garfall, Kelly Godby, Jens Hillengass, Leona Holmberg, Myo Htut, Carol Ann Huff, Malin Hultcrantz, Yubin Kang, Sarah Larson, Michaela Liedtke, Thomas Martin, James Omel, Douglas Sborov, Kenneth Shain, Keith Stockerl-Goldstein, Donna Weber, Ryan A. Berardi, Rashmi Kumar, and Shaji K. Kumar

The NCCN Guidelines for Multiple Myeloma provide recommendations for diagnosis, initial workup, treatment, follow-up, and supportive care for patients with various plasma cell neoplasms, including multiple myeloma. These NCCN Guidelines Insights highlight some of the important updates/changes specific to the treatment of patients with multiple myeloma in the 2022 version of the guidelines.

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NCCN Guidelines Insights: Multiple Myeloma, Version 1.2020

Featured Updates to the NCCN Guidelines

Shaji K. Kumar, Natalie S. Callander, Jens Hillengass, Michaela Liedtke, Muhamed Baljevic, Erica Campagnaro, Jorge J. Castillo, Jason C. Chandler, Robert F. Cornell, Caitlin Costello, Yvonne Efebera, Matthew Faiman, Alfred Garfall, Kelly Godby, Leona Holmberg, Myo Htut, Carol Ann Huff, Yubin Kang, Ola Landgren, Ehsan Malek, Thomas Martin, James Omel, Noopur Raje, Douglas Sborov, Seema Singhal, Keith Stockerl-Goldstein, Carlyn Tan, Donna Weber, Alyse Johnson-Chilla, Jennifer Keller, and Rashmi Kumar

The NCCN Guidelines for Multiple Myeloma provide recommendations for diagnosis, workup, treatment, follow-up, and supportive care for patients with monoclonal gammopathy of renal significance, solitary plasmacytoma, smoldering myeloma, and multiple myeloma. These NCCN Guidelines Insights highlight some of the important updates and changes in the 1.2020 version of the NCCN Guidelines for Multiple Myeloma.

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Multiple Myeloma, Version 3.2021, NCCN Clinical Practice Guidelines in Oncology

Shaji K. Kumar, Natalie S. Callander, Kehinde Adekola, Larry Anderson, Muhamed Baljevic, Erica Campagnaro, Jorge J. Castillo, Jason C. Chandler, Caitlin Costello, Yvonne Efebera, Matthew Faiman, Alfred Garfall, Kelly Godby, Jens Hillengass, Leona Holmberg, Myo Htut, Carol Ann Huff, Yubin Kang, Malin Hultcrantz, Sarah Larson, Michaela Liedtke, Thomas Martin, James Omel, Kenneth Shain, Douglas Sborov, Keith Stockerl-Goldstein, Donna Weber, Jennifer Keller, and Rashmi Kumar

Multiple myeloma is a malignant neoplasm of plasma cells that accumulate in bone marrow, leading to bone destruction and marrow failure. This manuscript discusses the management of patients with solitary plasmacytoma, smoldering multiple myeloma, and newly diagnosed multiple myeloma.

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Systemic Light Chain Amyloidosis, Version 2.2023, NCCN Clinical Practice Guidelines in Oncology

Shaji K. Kumar, Natalie S. Callander, Kehinde Adekola, Larry D. Anderson Jr., Muhamed Baljevic, Erica Campagnaro, Jorge J. Castillo, Caitlin Costello, Christopher D’Angelo, Srinivas Devarakonda, Noura Elsedawy, Alfred Garfall, Kelly Godby, Jens Hillengass, Leona Holmberg, Myo Htut, Carol Ann Huff, Malin Hultcrantz, Yubin Kang, Sarah Larson, Hans C. Lee, Michaela Liedtke, Thomas Martin, James Omel, Aaron Rosenberg, Douglas Sborov, Jason Valent, Ryan Berardi, and Rashmi Kumar

Primary systemic light chain amyloidosis (SLCA) is characterized by production of light chains that get converted to amyloid fibrils with an affinity for visceral organs and causing organ dysfunction. The therapy for SLCA is directed to recovering the function of the affected organs by targeting the abnormal plasma cell clone and slowing deposition of amyloid fibrils. The NCCN Guidelines for SLCA provide recommendations for workup, diagnosis, and treatment of primary as well as previously treated SLCA.

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Waldenström Macroglobulinemia/Lymphoplasmacytic Lymphoma, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology

Shaji K. Kumar, Natalie S. Callander, Kehinde Adekola, Larry D. Anderson Jr, Muhamed Baljevic, Rachid Baz, Erica Campagnaro, Jorge J. Castillo, Caitlin Costello, Christopher D’Angelo, Benjamin Derman, Srinivas Devarakonda, Noura Elsedawy, Alfred Garfall, Kelly Godby, Jens Hillengass, Leona Holmberg, Myo Htut, Carol Ann Huff, Malin Hultcrantz, Yubin Kang, Sarah Larson, Hans Lee, Michaela Liedtke, Thomas Martin, James Omel, Timothy Robinson, Aaron Rosenberg, Douglas Sborov, Mark A. Schroeder, Daniel Sherbenou, Attaya Suvannasankha, Jason Valent, Asya Nina Varshavsky-Yanovsky, Jenna Snedeker, and Rashmi Kumar

The treatment of Waldenström macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) has evolved to include several new options. The NCCN Guidelines for WM/LPL provide a framework on which to base decisions regarding diagnosis, treatment, assessment of response to treatment, and follow-up of both newly diagnosed and previously treated WM/LPL.

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Multiple Myeloma, Version 2.2024, NCCN Clinical Practice Guidelines in Oncology

Shaji K. Kumar, Natalie S. Callander, Kehinde Adekola, Larry D. Anderson Jr, Muhamed Baljevic, Rachid Baz, Erica Campagnaro, Jorge J. Castillo, Caitlin Costello, Christopher D’Angelo, Srinivas Devarakonda, Noura Elsedawy, Alfred Garfall, Kelly Godby, Jens Hillengass, Leona Holmberg, Myo Htut, Carol Ann Huff, Malin Hultcrantz, Yubin Kang, Sarah Larson, Hans C. Lee, Michaela Liedtke, Thomas Martin, James Omel, Timothy Robinson, Aaron Rosenberg, Douglas Sborov, Mark A. Schroeder, Daniel Sherbenou, Attaya Suvannasankha, Jason Valent, Asya Nina Varshavsky-Yanovsky, Rashmi Kumar, and Jenna Snedeker

The treatment of relapsed/refractory multiple myeloma (MM) has evolved to include several new options. These include new combinations with second generation proteasome inhibitors (PI); second generation immunomodulators, monoclonal antibodies, CAR T cells, bispecific antibodies, selinexor, venetoclax, and many others. Most patients with MM undergo several cycles of remissions and relapse, and therefore need multiple lines of combination therapies. Selecting treatment options for relapsed/refractory MM requires consideration of resistance status to specific classes, and patient-specific factors such as age and other comorbidities should be considered. The NCCN Guidelines for MM provide a framework on which to base decisions regarding workup, treatment, and follow-up of newly diagnosed and previously treated MM. This manuscript outlines the recommendations from NCCN Guidelines for MM specific to relapsed/refractory disease.