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Multiple Myeloma
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
There is no evidence for early treatment of patients with smoldering myeloma outside of a trial. Their rate of progression of smoldering myeloma is around 10% per year for 5 years but then reduces, and clearly a minority behave just as a benign MGUS without progression. They should be followed carefully, with serum protein electrophoresis and serum free light chains every 8 weeks.
Multiple myeloma
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
FDG PET-CT is more sensitive than plain film radiography and is also useful in investigating patients who are symptomatic with a negative skeletal survey (40,112). It is recommended in patients with smouldering myeloma (17). In patients with MGUS, it is invariably negative, so it has no role in this condition (107). The sensitivity of FDG PET-CT in detecting focal lesions in the spine and pelvis is broadly similar to MRI, but MRI is superior in detecting diffuse and variegated bone marrow infiltration (114–116). Metabolically active deposits irrespective of the underlying lytic lesion at CT are identified with a PET standard spatial resolution of about 5 mm. Renal failure and metallic bone implants are not a contraindication to the examination and, to date, bisphosphonate therapy has not been shown to affect FDG distribution. A summary of the technical requirements can be found in the recent IMWG consensus statement (113).
Central nervous system
Published in A Stewart Whitley, Jan Dodgeon, Angela Meadows, Jane Cullingworth, Ken Holmes, Marcus Jackson, Graham Hoadley, Randeep Kumar Kulshrestha, Clark’s Procedures in Diagnostic Imaging: A System-Based Approach, 2020
A Stewart Whitley, Jan Dodgeon, Angela Meadows, Jane Cullingworth, Ken Holmes, Marcus Jackson, Graham Hoadley, Randeep Kumar Kulshrestha
CT of the thorax and abdomen is frequently performed for surveillance and monitoring of malignancies, and integral to the interpretation of these examinations is an assessment of the thoracic and lumbar spine for possible metastases. Lesions of myeloma are better detected by CT and MRI than by plain radiographs, and in the UK cross-sectional imaging is advised by the National Institute for Health and Care Excellence (NICE) in newly diagnosed cases and in ‘smouldering myeloma’ where imaging has not been performed in the previous year [93]. NICE prefer MRI if not contraindicated, but CT may show early infractions slightly better [94]. CT may also be used for local anatomical assessment of spinal metastases with cord compressions where procedures such as vertebroplasty or surgery are being considered [95].
Identification of AL proteins from 10 λ-AL amyloidosis patients by mass spectrometry extracted from abdominal fat and heart tissue
Published in Amyloid, 2023
Julian Baur, Natalie Berghaus, Sarah Schreiner, Ute Hegenbart, Stefan O. Schönland, Sebastian Wiese, Stefanie Huhn, Christian Haupt
Our study includes 8 patients with dominant cardiac and 2 patients with dominant renal involvement (Table 1). The median age at diagnosis was 56.5 years (range: 34–80 years). Five patients had smouldering myeloma and one an overt multiple myeloma as underlying disease. The median dFLC was 374 mg/L (range: 96–1382 mg/mL). Seven of 8 patients with heart involvement showed very severe cardiac amyloidosis (cardiac stage IIIb) [3]. The 2 patients with kidney involvement did not have advanced renal stage (both I) [39]. All the fat aspirates used contained a large amount of amyloid (3+ or 4+) [40]. All precursor LCs overproduced in the patients belong to the λ isotype but originate from different IGLV gene segments, 1 from IGLV1-40 (FOR138), 5 from IGLV1-44 (FOR102, FOR107, FOR128, FOR142, FOR006), 2 from IGLV2-14 (FOR101, FOR159) and 2 from IGLV3-19 (FOR103, FOR005) (Table 1). These IGLV families are often associated with AL amyloidosis [6–9]. Due to the small total sample size no statement can be made about a preferential use of the IGLJ and IGLC gene segments.
Deep sequencing as an approach to understanding the complexity and improving the treatment of multiple myeloma
Published in Expert Review of Precision Medicine and Drug Development, 2020
Louis S. Williams, Jessica Caro, Beatrice Razzo, Eileen M. Boyle, Gareth J. Morgan
In order to investigate alterations in the myeloma genome over time, an early study of 28 paired low and high cytogenetic risk patients examined mutations and copy number abnormalities at various time points during treatment. The analysis revealed three paths of evolution: genomic stability, linear acquisition of genetic lesions, and branched evolution [53]. Additional evidence of branched clonal evolution emerged from a study in which the whole genomes of patients at various stages of disease progression, from MGUS to plasma cell leukemia, were sequenced and compared. Serial samples from the precursor stages of MM have shown that sub-clonal heterogeneity is present in patients with early MGUS and persists throughout the disease course. This subclonal heterogeneity can be present as both temporal and spatial events [2,48,54]. Whole-exome sequencing followed by single-cell multiplex PCR and copy number analysis allowed phylogenetic hierarchy of a t(11;14) MM and showed the persistence of a founder clone into the later stages of disease evolution [55]. The presence of early clonal heterogeneity and branching patterns of evolution in myeloma development has clinical implications for the treatment of smoldering myeloma, wherein intervention has been shown to delay time to development of symptomatic disease [56,57]
Smoldering multiple myeloma 40 years later: a story of unintended disease
Published in Expert Review of Hematology, 2021
The challenge of distinguishing imperceptible SMM grades from their clonal counterparts, MGUS and MM, has reached an unprecedented level becoming essentially experts’ prerogative. The term smoldering myeloma itself has become a double misnomer: the condition fails to fulfill the myeloma category lacking necessary disease attributes, and its clinical course no longer meets the definition of smoldering. It is paramount to recognize that the prototype SMM was introduced as a variant of MM, not as a distinct entity. The original message was simple and on-target: all asymptomatic patients who fulfill the criteria for MM ought to be observed to not overlook a non-progressive course akin to MGUS [1]. Instead, the myeloma community disposed of an indolent variant of MM and endorsed a new, unintended SMM category discounting the trouble of altered connotation. So what is in store for SMM? By defying inertia – the tenacious adversary of progress – the brave arena of monoclonal gammopathies could accomplish far-reaching goals should SMM be reclassified in line of the overwhelming pathogenetic, molecular and clinical evidence (Table 1). The streamlined classification could simplify and rectify premalignant PC dyscrasias to make them understandable and manageable in everyday oncology practice. Additionally, the frustrating ambiguity among practicing oncologists and SMM patients alike, with respect to the overly confounding disease identity, terminology, and risk stratification would cease to exist. Lastly, substituting an MGUS subcategory for SMM may spare the patients’ life-long apprehension of carrying a frightening, but not necessarily fatal diagnosis.