Elsevier

Leukemia Research

Volume 46, July 2016, Pages 85-88
Leukemia Research

Research paper
The clinical spectrum of IgM monoclonal gammopathy: A single center retrospective study of 377 patients

https://doi.org/10.1016/j.leukres.2016.05.002Get rights and content

Highlights

  • IgM monoclonal gammopathy contains a broad spectrum of diseases.

  • Levels of serum IgM can be used as one parameter for differential diagnosis.

  • Type of light chain can be used to help differential diagnosis among IgM monoclonal gammopathy.

  • The relationship between MGUS and some autoimmune diseases requires further investigation.

Abstract

Objectives

We retrospectively evaluated the clinical features, serum levels of IgM, and prevalence of IgM related diseases in patients with serum immunofixation electrophoresis (sIFE) confirmed IgM monoclonal gammopathy at our center.

Methods

We included patients with sIFE confirmed IgM monoclonal gammopathy between January 2008 and December 2014 in this retrospective study. We evaluated clinical data, sIFE, serum IgM levels, and diagnosis.

Results

In total, 7107 patients had sIFE confirmed monoclonal gammopathy, with 377 (5.3%) patients having the IgM type. The median age was 62 years (range, 19–105 years). The median level of serum IgM is 8.3 g/L (range, 0.24–150 g/L). The diagnosis included monoclonal gammopathy of undetermined significance (MGUS, 157 patients, 41.6%), Waldenstrom macroglobulinemia (WM, 105 patients, 27.9%), B cell non-Hodgkin’s lymphoma (69 patients, 18.3%), primary cold agglutinin disease (pCAD, 16 patients, 4.2%), primary amyloidosis (14 patients, 3.7%), cryoglobulinaemia (six patients, 1.6%), IgM MGUS associated neuropathy (five patients, 1.3%), multiple myeloma (three patients, 0.8%), and POEMS syndrome (two patients, 0.5%). Levels of serum IgM > 15.5 g/L were 80.6% sensitive and 89.2% specific for the diagnosis of WM. Kappa type light chain indicated the diagnosis of WM, pCAD, IgM MGUS associated neuropathy and cryoglobulinaemia, while lambda type light chain indicated POEMS and amyloidosis. There were 41/157 (26.1%) MGUS patients diagnosed with complications due to IgM-unrelated autoimmune diseases.

Conclusion

IgM monoclonal gammopathy contains a broad spectrum of diseases. Levels of serum IgM and the type of light chain can be used to help with differential diagnosis. The association between MGUS and some autoimmune diseases requires further investigation.

Introduction

The presence of IgM monoclonal gammopathy can occur in a broad spectrum of diseases, including Waldenstrom macroglobulinemia (WM), various B cell non-Hodgkin’s lymphoma (NHL), multiple myeloma (MM), primary amyloidosis (AL), monoclonal gammopathy of undetermined significance (MGUS), and so on. The clinical manifestations of IgM monoclonal gammopathy related diseases vary widely from quiet lesions to aggressive, and potentially lethal, diseases [1], [2], [3]. This rare condition is often misdiagnosed due to rarity and heterogeneity. To date, there is little information regarding IgM monoclonal gammopathy related diseases. The prevalence, clinical manifestations, serum level of IgM, and type of light chain in each type of IgM related disease has never been reported comprehensively. In the current study, we retrospectively evaluated the patients with serum immunofixation electrophoresis (sIFE) confirmed IgM monoclonal gammopathy in a single consecutive cohort. We focused on the clinical features, serum levels of IgM, and the prevalence of IgM related diseases.

Section snippets

Patients

We included patients with sIFE confirmed IgM monoclonal gammopathy between January 2008 and December 2014 at Peking Union Medical College Hospital in this retrospective study. We collected clinical data, including age, sex, chief complaint, physical examination, sIFE, and routine laboratory analyses. Routine laboratory analyses included evaluation of complete blood count, levels of lactic dehydrogenase (LDH), levels of serum IgM. We also collected 18F Fluorodeoxyglucose positron emission

General characteristics

In total, there were 7107 patients with sIFE confirmed monoclonal gammopathy between January 2008 and December 2014 at Peking Union Medical College Hospital, with 377 patients having the IgM type (5.3%). That included 233 male patients (61.8%) and 144 female patients (38.2%). The median age of patients was 62 years (range, 19–105 years); 38 patients (10.1%) were younger than 40 years, whereas 291 patients (77.2%) were more than 50 years old, and 98 patients (26.0%) were aged 70 years or older.

Discussion

IgM monoclonal gammopathy is most frequently found in WM and IgM MGUS. The clinical spectrum, underlying disease, and prevalence of IgM monoclonal gammopathy have never been reported. This single-center study includes 7107 patients with sIFE confirmed monoclonal gammopathy, with IgM type accounting for 5.3%. That rate is similar to those reported in African American, native African, and Asian populations [11], [12], [13], while it is much lower than those reported in Caucasians [14]. In the

Conflict of interest

The authors report no conflicts of interest related to the content of this paper.

Acknowledgement

This study was not supported by any foundation.

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