From the Guidelines
The patient has a high-risk monoclonal gammopathy that is classified as either high-risk MGUS (monoclonal gammopathy of undetermined significance) or smoldering multiple myeloma, with a significantly abnormal free light chain ratio and expression of CD45, CD138, CD38, CD56, and CD117, indicating plasma cell dyscrasia. The bone marrow biopsy shows less than 10% plasma cells, but these cells are monoclonal with an abnormal kappa:lambda light chain ratio of 5.1:1, which is consistent with plasma cell dyscrasia 1. The patient's free light chain ratio is significantly abnormal, placing her outside the low-risk category.
- A PET/CT whole body scan is appropriate to determine if there are any bone lesions, which would help differentiate between MGUS and myeloma 1.
- Even with <10% plasma cells in the bone marrow, the presence of more than one plasma cell lesion on imaging would qualify for a multiple myeloma diagnosis according to current criteria 1. The patient's normal kidney function (creatinine and eGFR) is reassuring, though she does have lymphopenia and monocytopenia.
- Close monitoring is essential as high-risk MGUS has approximately a 10% per year risk of progression to symptomatic multiple myeloma 1. It is crucial to track the progression of myeloma disease and response to treatment through regular assessments of the M-protein component in serum and urine, as well as serum free light chain (FLC) assay 1.
From the Research
Clinical Significance
The clinical significance of a 36-year-old female with scattered interstitial T-cells and B-cells, a cytoplasmic kappa:lambda light chain ratio of 5.1:1, and expression of dim CD45, CD138, CD38, CD56, and CD117, in the context of monoclonal plasma cells, can be understood by considering the following points:
- The presence of monoclonal plasma cells is a key feature of multiple myeloma, a type of hematologic malignancy that accounts for approximately 10% of such cancers 2, 3, 4, 5, 6.
- The diagnosis of multiple myeloma requires ≥10% clonal bone marrow plasma cells or a biopsy-proven plasmacytoma plus evidence of one or more multiple myeloma defining events (MDE) 2, 3, 4, 5, 6.
- The patient's normal creatinine levels and elevated estimated Glomerular Filtration Rate (eGFR) suggest that renal function is not significantly impaired, which is an important consideration in the diagnosis and management of multiple myeloma 2, 3, 4, 5, 6.
- The low absolute lymphocyte and monocyte counts may indicate immunosuppression or bone marrow suppression, which can be associated with multiple myeloma or its treatment 2, 3, 4, 5, 6.
Risk Stratification
The patient's risk stratification can be determined by considering the following factors:
- The presence of certain cytogenetic abnormalities, such as del(17p), t(4;14), t(14;16), t(14;20), gain 1q, or p53 mutation, which are associated with high-risk multiple myeloma 3, 4, 5.
- The patient's age, performance status, and other clinical factors, which can influence the choice of treatment and prognosis 2, 3, 4, 5, 6.
Treatment Options
The treatment options for the patient can be considered based on the following:
- Induction therapy with bortezomib, lenalidomide, and dexamethasone (VRd) or other regimens, followed by autologous stem cell transplantation (ASCT) in eligible patients 2, 3, 4, 5, 6.
- Maintenance therapy with lenalidomide or other agents to prevent relapse and improve survival 2, 3, 4, 5, 6.
- The use of novel agents, such as daratumumab, carfilzomib, and ixazomib, in combination with other therapies to improve outcomes 3, 4, 5.