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IgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders
A subset of patients with immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (MGUS) develop IgM-related disorders (IgM-RD) including peripheral neuropathy, cryoglobulinemia and/or cold agglutinin disease (CAD). We examined the clinical and bone marrow pathologic findings in 19...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Fondazione Ferrata Storti
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10543195/ https://www.ncbi.nlm.nih.gov/pubmed/37021542 http://dx.doi.org/10.3324/haematol.2022.282389 |
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author | Bruehl, Frido K. Mannion, Peter Barbato, Elisha Nakashima, Megan O. Cook, James R. |
author_facet | Bruehl, Frido K. Mannion, Peter Barbato, Elisha Nakashima, Megan O. Cook, James R. |
author_sort | Bruehl, Frido K. |
collection | PubMed |
description | A subset of patients with immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (MGUS) develop IgM-related disorders (IgM-RD) including peripheral neuropathy, cryoglobulinemia and/or cold agglutinin disease (CAD). We examined the clinical and bone marrow pathologic findings in 191 IgM MGUS patients (2016 World Health Oragnization criteria). Clonal plasma cells were identified in 41 of 171 (24%) cases by immunohistochemistry (IHC) and clonal B cells in 43 of 157 (27%). IgM-RD was identified in 82 (43%) cases, including peripheral neuropathy (n=67, 35%), cryoglobulinemia (n=21, 11%), and CAD (n=10, 5%). Cases of CAD showed distinctive features including lack of MYD88 mutations (P=0.048), supporting the concept of primary CAD as a distinct clinicopathologic disorder. Following exclusion of CAD, comparison of the remaining cases with (n=72) or without (n=109) IgM-RD showed IgM-RD to be more frequent in men than women (P=0.02) and to be more highly associated with MYD88 L265P (P=0.011). Cases with and without IgM-RD otherwise showed similar features including serum IgM concentrations, presence of lymphoid aggregates, clonal B cells by flow cytometry or clonal plasma cells by IHC. No differences were observed in overall survival between cases with and without IgM-RD. No cases in this series met criteria for plasma cell type IgM MGUS as defined in the 2022 International Consensus Classification of lymphoid neoplasms. These results show IgM-RD to be common in patients with IgM MGUS. While CAD shows distinctive features, the remaining cases of IgM-RD largely show pathologic findings similar to IgM MGUS without IgM-RD. |
format | Online Article Text |
id | pubmed-10543195 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Fondazione Ferrata Storti |
record_format | MEDLINE/PubMed |
spelling | pubmed-105431952023-10-03 IgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders Bruehl, Frido K. Mannion, Peter Barbato, Elisha Nakashima, Megan O. Cook, James R. Haematologica Article - Plasma Cell Disorders A subset of patients with immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (MGUS) develop IgM-related disorders (IgM-RD) including peripheral neuropathy, cryoglobulinemia and/or cold agglutinin disease (CAD). We examined the clinical and bone marrow pathologic findings in 191 IgM MGUS patients (2016 World Health Oragnization criteria). Clonal plasma cells were identified in 41 of 171 (24%) cases by immunohistochemistry (IHC) and clonal B cells in 43 of 157 (27%). IgM-RD was identified in 82 (43%) cases, including peripheral neuropathy (n=67, 35%), cryoglobulinemia (n=21, 11%), and CAD (n=10, 5%). Cases of CAD showed distinctive features including lack of MYD88 mutations (P=0.048), supporting the concept of primary CAD as a distinct clinicopathologic disorder. Following exclusion of CAD, comparison of the remaining cases with (n=72) or without (n=109) IgM-RD showed IgM-RD to be more frequent in men than women (P=0.02) and to be more highly associated with MYD88 L265P (P=0.011). Cases with and without IgM-RD otherwise showed similar features including serum IgM concentrations, presence of lymphoid aggregates, clonal B cells by flow cytometry or clonal plasma cells by IHC. No differences were observed in overall survival between cases with and without IgM-RD. No cases in this series met criteria for plasma cell type IgM MGUS as defined in the 2022 International Consensus Classification of lymphoid neoplasms. These results show IgM-RD to be common in patients with IgM MGUS. While CAD shows distinctive features, the remaining cases of IgM-RD largely show pathologic findings similar to IgM MGUS without IgM-RD. Fondazione Ferrata Storti 2023-04-06 /pmc/articles/PMC10543195/ /pubmed/37021542 http://dx.doi.org/10.3324/haematol.2022.282389 Text en Copyright© 2023 Ferrata Storti Foundation https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution Noncommercial License (by-nc 4.0) which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited. |
spellingShingle | Article - Plasma Cell Disorders Bruehl, Frido K. Mannion, Peter Barbato, Elisha Nakashima, Megan O. Cook, James R. IgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders |
title | IgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders |
title_full | IgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders |
title_fullStr | IgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders |
title_full_unstemmed | IgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders |
title_short | IgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders |
title_sort | igm monoclonal gammopathy of undetermined significance: clinicopathologic features with and without igm-related disorders |
topic | Article - Plasma Cell Disorders |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10543195/ https://www.ncbi.nlm.nih.gov/pubmed/37021542 http://dx.doi.org/10.3324/haematol.2022.282389 |
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