Prevalence, clinical aspects, and natural history of IgM MGUS

McMaster, Mary L.; Landgren, Ola

doi:10.1002/cyto.b.20550

Cited by 21 publications

(11 citation statements)

References 56 publications

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“…The monoclonal IgM proteins are found in 1 of 600 patients older than 50 years . The overall age‐standardized rate of WM is only 5.5 per 1 million persons per year .…”

Section: Diagnosismentioning

confidence: 99%

Waldenström macroglobulinemia: 2015 update on diagnosis, risk stratification, and management

Gertz

2015

American J Hematol

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Disease Overview: Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity. Diagnosis: Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in more than 90% of patients. Risk Stratification: Age, hemoglobin level, platelet count, β2 microglobulin, and monoclonal IgM concentrations are characteristics required for prognosis. Risk‐Adapted Therapy: Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Rituximab‐based therapy is used in virtually all US patients with WM and can be combined with alkylating agent or purine nucleoside analog (or both). The preferred Mayo Clinic nonstudy therapeutic induction is rituximab, cyclophosphamide, and dexamethasone. Future stem cell transplantation should be considered in induction therapy selection. Management of Refractory Disease: Bortezomib, thalidomide, everolimus, ibrutinib, carfilzomib, lenalidomide, and bendamustine have all been shown to have activity in WM. Given WM's natural history, reduction of complications will be a priority for future treatment trials. Am. J. Hematol. 90:347–354, 2015. © 2015 Wiley Periodicals, Inc.

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“…The monoclonal IgM proteins are found in 1 of 600 patients older than 50 years . The overall age‐standardized rate of WM is only 5.5 per 1 million persons per year .…”

Section: Diagnosismentioning

confidence: 99%

Waldenström macroglobulinemia: 2015 update on diagnosis, risk stratification, and management

Gertz

2015

American J Hematol

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“…4 Distribution of a MBL subtypes by immunophenotyping (n=63) and b monoclonal immunoglobulin isotypes by serum immunofixation electrophoresis (n=150) Prevalence of MGUS has been reported in up to 5.9 % of individuals in general population-based screenings and therefore deviates from the prevalence of 9.8 % in our hospital cohort [21]. In accordance with the literature and similar to the findings in MBL, we found an increasing prevalence of MGUS with age [21]. Interestingly, IgM+ MGUS were considerably overrepresented (24.7 %) as compared to previous studies [10].…”

Section: Discussionmentioning

confidence: 56%

Hospital population screening reveals overrepresentation of CD5− monoclonal B-cell lymphocytosis and monoclonal gammopathy of undetermined significance of IgM type

et al. 2015

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Monoclonal B-cell lymphocytosis (MBL) and monoclonal gammopathy of undetermined significance (MGUS) result from clonal expansions of mature B or plasma cells. Here, we set out to determine the immunophenotypic/monoclonal immunoglobulin (M protein) features and co-prevalence of MBL and MGUS in a hospital-based cohort of 1909 non-hematooncological patients. Of the evaluable cases, 3.8 % showed evidence for MBL by immunophenotyping, while 9.8 % were screened positive for M protein by immunofixation. With six concomitant cases (0.4 %), MBL and MGUS were not statistically associated. At least in two of these coincident cases, MBL and MGUS were of different clonal origin since both clones had divergent light chain restriction. CD5(-) MBL (57.1 %) and IgM+ MGUS (24.7 %) were strikingly overrepresented compared to population-based screenings and did not progress to overt lymphoma or myeloma during the observation period (mean follow-up of 117 weeks or 110 weeks, respectively). Prevalence and phenotypes suggest that a substantial proportion of incidental MBL and MGUS in hospitalized patients may be attributed to transiently expanded B-cell clones in the context of disease-related immune stimulation rather than reflecting veritable precursors of clonal B-cell malignancies.

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“…Monoclonal IgM proteins are found in 1 of 600 persons older than 50 years . Far more patients have IgM MGUS than have WM.…”

Section: Diagnosismentioning

confidence: 99%

Waldenström macroglobulinemia: 2019 update on diagnosis, risk stratification, and management

Gertz

2018

American J Hematol

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Disease Overview Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity. Diagnosis Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in >90% of patients and is found in the majority of IgM monoclonal gammopathy of undetermined significance patients. Risk Stratification Age, hemoglobin level, platelet count, β2 microglobulin, and monoclonal IgM concentrations are characteristics that are predictive of outcomes. Risk‐Adapted Therapy Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Rituximab‐monotherapy is inferior to regimens that combine it with bendamustine, an alkylating agent, a proteosome inhibitor, or ibrutinib. Purine nucleoside analogs are active but usage is declining for less toxic alternatives. The preferred Mayo Clinic induction is rituximab and bendamustine. Potential for stem cell transplantation should be considered in selected younger patients. Management of Refractory Disease Bortezomib, fludarabine, thalidomide, everolimus, ibrutinib, carfilzomib, lenalidomide, and bendamustine have all been shown to have activity in relapsed WM. Given WM's natural history, reduction of therapy toxicity is an important part of treatment selection.

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Prevalence, clinical aspects, and natural history of IgM MGUS

Cited by 21 publications

References 56 publications

Waldenström macroglobulinemia: 2015 update on diagnosis, risk stratification, and management

Waldenström macroglobulinemia: 2015 update on diagnosis, risk stratification, and management

Hospital population screening reveals overrepresentation of CD5− monoclonal B-cell lymphocytosis and monoclonal gammopathy of undetermined significance of IgM type

Waldenström macroglobulinemia: 2019 update on diagnosis, risk stratification, and management

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