The evaluation and management of monoclonal gammopathy of renal significance and monoclonal gammopathy of neurological significance

Castillo, Jorge J.; Callander, Natalie S.; Baljevic, Muhamed; Sborov, Douglas W.; Kumar, Shaji

doi:10.1002/ajh.26155

Cited by 21 publications

(14 citation statements)

References 58 publications

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“…MGNS is defined as neuropathy caused by a monoclonal protein, and often requires neurology specialist input for diagnosis 28 . Peripheral neuropathy is a frequent finding in MG patients, with up to 30-50% prevalence in IgM MG patients, 5% in IgG MG, 15% in IgA MG 33 .…”

Section: Paraprotein-mediated Tissue Damagementioning

confidence: 99%

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Monoclonal gammopathy of increasing significance: time to screen?

et al. 2022

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Monoclonal gammopathy (MG) is a frequently detected clonal B-cell or plasma cell disorder. Importantly, every multiple myeloma (MM) case is preceded by MG. Although clinical algorithms now allow earlier treatment of patients with biomarkers of malignancy before MM-induced tissue damage (CRAB) occurs, most patients are still diagnosed late. It is important to revisit how MG should be managed in clinical practice and whether screening is required. As the prevalence of MG and other medical co-morbidities both rise with increasing age, the degree of contribution of MG to disease states other than malignant progression is often unclear. This can lead to monitoring lapses and under recognition of the organ dysfunction that can occur with monoclonal gammopathy of clinical significance (MGCS). Therefore, models of progression to MM and/or MGCS require further refinement. Whilst currently MG is detected incidentally, a case for screening has been made with ongoing studies in this area. Screening has the potential benefit of earlier detection and prevention of both MGCS and delayed MM presentations, but important drawbacks include the psychosocial impact on individuals and resource burden on healthcare services. MG terminology should transition alongside our increasing understanding of the condition and genomic characterisation that have already begun to revise the MG nomenclature. The biology of MG has been poorly understood and is often inferred from the biology of MM, which is unhelpful. We review the literature and case for MG screening in this paper. In particular, we highlight areas that require focus to establish screening for MG.

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Section: Paraprotein-mediated Tissue Damagementioning

confidence: 99%

“…Evidence suggests that treatment of the underlying plasma or B-cell clonal disorder can ameliorate symptoms and prevent irreversible organ damage in MGCS 28 . Further studies, both large-scale epidemiological and biological, are required to fully understand the aetiology of MG-related disorders.…”

Section: Paraprotein-mediated Tissue Damagementioning

confidence: 99%

Monoclonal gammopathy of increasing significance: time to screen?

et al. 2022

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“…The timely administration of anti-clonal therapy may help to avoid this inexorable course in some cases. The goal of therapy should focus on preventing further renal damage by the M-protein and reaching a hematologic response because hematologic responses are a prerequisite to achieving renal responses [ 62 , 63 , 64 , 65 , 66 , 67 ].…”

Section: Monoclonal Gammopathies Of Renal Significancementioning

confidence: 99%

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

Ríos-Tamayo

Lahuerta

Martínez‐López

et al. 2022

Cancers

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Monoclonal gammopathies of clinical significance (MGCSs) represent a group of diseases featuring the association of a nonmalignant B cells or plasma cells clone, the production of an M-protein, and singularly, the existence of organ damage. They present a current framework that is difficult to approach from a practical clinical perspective. Several points should be addressed in order to move further toward a better understanding. Overall, these entities are only partially included in the international classifications of diseases. Its definition and classification remain ambiguous. Remarkably, its real incidence is unknown, provided that a diagnostic biopsy is mandatory in most cases. In fact, amyloidosis AL is the final diagnosis in a large percentage of patients with renal significance. On the other hand, many of these young entities are syndromes that are based on a dynamic set of diagnostic criteria, challenging a timely diagnosis. Moreover, a specific risk score for progression is lacking. Despite the key role of the clinical laboratory in the diagnosis and prognosis of these patients, information about laboratory biomarkers is limited. Besides, the evidence accumulated for many of these entities is scarce. Hence, national and international registries are stimulated. In particular, IgM MGCS deserves special attention. Until now, therapy is far from being standardized, and it should be planned on a risk and patient-adapted basis. Finally, a comprehensive and coordinated multidisciplinary approach is needed, and specific clinical trials are encouraged.

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“…Monoclonal gammopathy of neurologic significance (MGNS) may be diagnosed if the following factors are present: symmetric sensory deficits, slow progression, length dependence, strongly positive anti-MAG antibodies, and prominent demyelination on EMG. Features less consistent with MGNS (asymmetry, motor-predominant, rapidly progressive, length independent, negative MAG-antibodies, or axonal nerve conduction) should prompt a work-up for other diseases that may be associated with an IgM paraprotein and peripheral neuropathy, such as AL amyloidosis, POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes), cryoglobulinemia, and Bing-Neel syndrome ( 8 ).…”

Section: Clinical Presentation and Diagnosismentioning

confidence: 99%

Diagnosis and management of AL amyloidosis due to B-cell non-Hodgkin lymphoma

Berkowitz

Dittus

2022

Front. Oncol.

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Immunoglobulin light chain (AL) amyloidosis may be caused by a B-cell non-Hodgkin lymphoma (NHL) rather than a plasma cell neoplasm in rare cases, which presents unique diagnostic and management considerations. NHL associated with AL will often have an IgM paraprotein; thus, this disease is termed IgM-related AL amyloidosis (IgM AL). The clinical presentation of IgM AL is more likely to involve the lungs, peripheral nerves, and soft tissue; cardiac involvement is less common. Patients with IgM AL amyloidosis should undergo a lymphoma-directed work-up including evaluation for nodal and extranodal disease. Additionally, patients with an IgM paraproteinemia should be screened for AL amyloidosis through history and physical examination. Treatment regimens active against underlying lymphoma, rather than plasma cell-directed regimens, are recommended. Historical response rates in IgM AL have been poor; prospective studies of novel antineoplastic regimens may improve treatment outcomes.

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The evaluation and management of monoclonal gammopathy of renal significance and monoclonal gammopathy of neurological significance

Cited by 21 publications

References 58 publications

Monoclonal gammopathy of increasing significance: time to screen?

Monoclonal gammopathy of increasing significance: time to screen?

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

Diagnosis and management of AL amyloidosis due to B-cell non-Hodgkin lymphoma

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