ABSTRACT
In this issue, the British Society for Haematology presents guidelines for the diagnosis and management of patients with smouldering multiple myeloma (SMM). The authors provide a practical, evidence-based approach to managing these patients. Key questions remain yet unsolved. Commentary on: Hughes et al. Diagnosis and management of smouldering myeloma: A British Society for Haematology Good Practice Paper. Br J Haematol 2024;204:1193-1206.
Subject(s)
Monoclonal Gammopathy of Undetermined Significance , Multiple Myeloma , Smoldering Multiple Myeloma , Humans , Multiple Myeloma/diagnosis , Multiple Myeloma/therapy , Monoclonal Gammopathy of Undetermined Significance/diagnosis , Smoldering Multiple Myeloma/therapy , Disease ProgressionABSTRACT
Multiple myeloma is a bone marrow-based plasma cell tumour that develops from asymptomatic pre-cursor conditions smouldering myeloma and monoclonal gammopathy of uncertain significance and all are characterised by the presence of a monoclonal protein in the blood. Diagnosis and distinction between these conditions is based on blood tests, the bone marrow biopsy and cross sectional imaging. There are various risk stratification models that group patients with smouldering myeloma into risk groups based on risk of progression to symptomatic disease. Management is mainly observational for patients with smouldering myeloma although clinical trials for high-risk disease may be available. Restaging is required if evidence for progression.
Subject(s)
Hematology , Monoclonal Gammopathy of Undetermined Significance , Multiple Myeloma , Smoldering Multiple Myeloma , Humans , Multiple Myeloma/diagnosis , Multiple Myeloma/therapy , Multiple Myeloma/pathology , Plasma Cells/pathology , Monoclonal Gammopathy of Undetermined Significance/diagnosis , Monoclonal Gammopathy of Undetermined Significance/therapy , Monoclonal Gammopathy of Undetermined Significance/pathology , Smoldering Multiple Myeloma/diagnosis , Smoldering Multiple Myeloma/therapy , Smoldering Multiple Myeloma/pathology , Disease ProgressionABSTRACT
Smoldering multiple myeloma (SMM) is an intermediate clinical stage in the spectrum of monoclonal plasma cell disorders. It represents a heterogeneous clinically defined condition in which some patients (approximately 50%) have monoclonal gammopathy of undetermined significance (premalignancy), and some (approximately 50%) have multiple myeloma (biologic malignancy). Using specific prognostic factors, patients with SMM, in whom malignant transformation has already likely occurred, can be identified. These patients are considered to have high-risk SMM. Patients with newly diagnosed high-risk SMM are candidates for early intervention with lenalidomide or lenalidomide plus dexamethasone for 2 years, or enrollment in clinical trials.
Subject(s)
Monoclonal Gammopathy of Undetermined Significance , Multiple Myeloma , Smoldering Multiple Myeloma , Humans , Smoldering Multiple Myeloma/diagnosis , Smoldering Multiple Myeloma/therapy , Lenalidomide/therapeutic use , Disease Progression , Multiple Myeloma/diagnosis , Multiple Myeloma/therapy , Monoclonal Gammopathy of Undetermined Significance/pathology , Risk FactorsABSTRACT
The approach to patients with high-risk smoldering multiple myeloma (SMM) varies among clinicians; while some advocate early intervention, others reserve treatment at progression to multiple myeloma (MM). We aimed to describe the myeloma-defining events (MDEs) and clinical presentations leading to MM diagnosis among SMM patients seen at our institution. We included 406 patients diagnosed with SMM between 2013-2022, seen at Mayo Clinic, Rochester, MN. The 2018 Mayo 20/2/20 criteria were used for risk stratification. Median follow-up was 3.9 years. Among high-risk patients who did not receive treatment in the SMM phase (n = 71), 51 progressed by last follow-up; the MDEs included: bone lesions (37%), anemia (35%), hypercalcemia (8%), and renal failure (6%); 24% met MM criteria based on marrow plasmacytosis (≥60%) and/or free light chain ratio (>100); 45% had clinically significant MDEs (hypercalcemia, renal insufficiency, and/or bone lesions). MM diagnosis was made based on surveillance labs/imaging(45%), testing obtained due to provider suspicion for progression (14%), bone pain (20%), and hospitalization/ED presentations due to MM complications/symptoms (4%). The presentation was undocumented in 14%. A high proportion (45%) of patients with high-risk SMM on active surveillance develop end-organ damage at progression. About a quarter of patients who progress to MM are not diagnosed based on routine interval surveillance testing.
Subject(s)
Hypercalcemia , Multiple Myeloma , Smoldering Multiple Myeloma , Humans , Smoldering Multiple Myeloma/diagnosis , Smoldering Multiple Myeloma/therapy , Disease Progression , Multiple Myeloma/complications , Multiple Myeloma/diagnosis , Multiple Myeloma/epidemiology , Immunoglobulin Light Chains , Risk FactorsABSTRACT
Precursor diseases of multiple myeloma (MM) are monoclonal gammopathy of uncertain significance and smoldering MM. While it is well known that a percentage of those affected by these conditions will progress to MM, it is difficult to predict who will progress and when, and guidelines for screening for these conditions are lacking. Moreover, there are various models for risk stratification, though there are ongoing efforts to improve these models in order to predict who may benefit from treatment. Finally, there are various clinical trials, both past and ongoing, expanding the scope of possible treatment options for precursor diseases.