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Monoclonal Gammopathy of Undetermined Significance (MGUS)—Not So Asymptomatic after All
Open AccessReview

Immunoglobulin M Paraproteinaemias

1
School of Medicine, University of Aberdeen, Aberdeen AB25 2ZN, UK
2
Aberdeen Royal Infirmary, NHS Grampian, Scotland AB25 2ZD, UK
3
Department of Haematology-Oncology, National University Cancer Institute, National University Health System, Singapore 119228, Singapore
4
Cancer Science Institute of Singapore, National University of Singapore, Singapore 117599, Singapore
5
Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore 119077, Singapore
*
Author to whom correspondence should be addressed.
Cancers 2020, 12(6), 1688; https://doi.org/10.3390/cancers12061688
Received: 6 May 2020 / Revised: 14 June 2020 / Accepted: 23 June 2020 / Published: 25 June 2020
(This article belongs to the Special Issue The Asymptomatic Version of Myeloma: MGUS and Smoldering Myeloma)
Monoclonal paraproteinaemia is an increasingly common reason for referral to haematology services. Paraproteinaemias may be associated with life-threatening haematologic malignancies but can also be an incidental finding requiring only observation. Immunoglobulin M (IgM) paraproteinaemias comprise 15–20% of monoclonal proteins but pose unique clinical challenges. IgM paraproteins are more commonly associated with lymphoplasmacytic lymphoma than multiple myeloma and can occur in a variety of other mature B-cell neoplasms. The large molecular weight of the IgM multimer leads to a spectrum of clinical manifestations more commonly seen with IgM paraproteins than others. The differential diagnosis of B-cell and plasma cell dyscrasias associated with IgM gammopathies can be challenging. Although the discovery of MYD88 L265P and other mutations has shed light on the molecular biology of IgM paraproteinaemias, clinical and histopathologic findings still play a vital role in the diagnostic process. IgM secreting clones are also associated with a number of “monoclonal gammopathy of clinical significance” entities. These disorders pose a novel challenge from both a diagnostic and therapeutic perspective. In this review we provide a clinical overview of IgM paraproteinaemias while discussing the key advances which may affect how we manage these patients in the future. View Full-Text
Keywords: immunoglobulin M; paraproteinaemia; Waldenstrom macroglobulinaemia; multiple myeloma; lymphoma immunoglobulin M; paraproteinaemia; Waldenstrom macroglobulinaemia; multiple myeloma; lymphoma
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Girard, L.-P.; Soekojo, C.Y.; Ooi, M.; Poon, L.M.; Chng, W.-J.; de Mel, S. Immunoglobulin M Paraproteinaemias. Cancers 2020, 12, 1688.

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