A rare case of IgE kappa monoclonal gammopathy of undetermined significance identified in a Swedish female

Factor X deficiency is a rare coagulopathy that can be inherited or acquired. Acquired factor X deficiency has been associated with plasma cell dyscrasias, amyloids, and use of vitamin K antagonists. Of plasma cell dyscrasias, most cases in the literature have been associated with multiple myeloma with or without concomitant AL amyloidosis. Here, we present a rare case of acquired isolated factor X deficiency in an elderly patient with immunoglobulin A (Ig A) monoclonal gammopathy of undetermined significance (MGUS). Herein, we highlight a rare cause of acquired factor X deficiency, and we hope to contribute to the growing literature of plasma cell dyscrasias associated with factor X deficiency.

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Faculty Opinions recommendation of Clues to pathogenesis of Waldenström macroglobulinemia and immunoglobulin M monoclonal gammopathy of undetermined significance provided by analysis of immunoglobulin heavy chain gene rearrangement and clustering of B-cell receptors.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.720399520.793540227 ◽

2017 ◽

Author(s):

Stephen Ansell

Keyword(s):

Heavy Chain ◽

Monoclonal Gammopathy ◽

Gene Rearrangement ◽

Immunoglobulin M ◽

B Cell Receptors ◽

Chain Gene ◽

Heavy Chain Gene ◽

Immunoglobulin Heavy Chain Gene ◽

Chain Gene Rearrangement ◽

Undetermined Significance

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Comparison of Monoclonal Gammopathies Linked to Poliovirus or Coxsackievirus vs. Other Infectious Pathogens

Cells ◽

10.3390/cells10020438 ◽

2021 ◽

Vol 10 (2) ◽

pp. 438

Author(s):

Jean Harb ◽

Nicolas Mennesson ◽

Cassandra Lepetit ◽

Maeva Fourny ◽

Margaux Louvois ◽

...

Keyword(s):

Multiple Myeloma ◽

B Cell ◽

Monoclonal Gammopathy ◽

Coat Proteins ◽

Chronic Stimulation ◽

B Cell Epitopes ◽

Monoclonal Immunoglobulins ◽

Self Antigen ◽

Coxsackievirus B1 ◽

Undetermined Significance

Chronic stimulation by infectious pathogens or self-antigen glucosylsphingosine (GlcSph) can lead to monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma (MM). Novel assays such as the multiplex infectious antigen microarray (MIAA) and GlcSph assays, permit identification of targets for >60% purified monoclonal immunoglobulins (Igs). Searching for additional targets, we selected 28 purified monoclonal Igs whose antigen was not represented on the MIAA and GlcSph assays; their specificity of recognition was then analyzed using microarrays consisting of 3760 B-cell epitopes from 196 pathogens. The peptide sequences PALTAVETG and PALTAAETG of the VP1 coat proteins of human poliovirus 1/3 and coxsackievirus B1/B3, respectively, were specifically recognized by 6/28 monoclonal Igs. Re-analysis of patient cohorts showed that purified monoclonal Igs from 10/155 MGUS/SM (6.5%) and 3/147 MM (2.0%) bound to the PALTAVETG or PALTAAETG epitopes. Altogether, PALTAV/AETG-initiated MGUS are not rare and few seem to evolve toward myeloma.

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Iceland screens, treats, or prevents multiple myeloma (iStopMM): a population-based screening study for monoclonal gammopathy of undetermined significance and randomized controlled trial of follow-up strategies

Blood Cancer Journal ◽

10.1038/s41408-021-00480-w ◽

2021 ◽

Vol 11 (5) ◽

Author(s):

Sæmundur Rögnvaldsson ◽

Thorvardur Jon Love ◽

Sigrun Thorsteinsdottir ◽

Elín Ruth Reed ◽

Jón Þórir Óskarsson ◽

...

Keyword(s):

Multiple Myeloma ◽

Early Treatment ◽

Monoclonal Gammopathy ◽

Controlled Trial ◽

Participation Rate ◽

Population Based ◽

Early Therapy ◽

Screening Study ◽

Undetermined Significance

AbstractMonoclonal gammopathy of undetermined significance (MGUS) precedes multiple myeloma (MM). Population-based screening for MGUS could identify candidates for early treatment in MM. Here we describe the Iceland Screens, Treats, or Prevents Multiple Myeloma study (iStopMM), the first population-based screening study for MGUS including a randomized trial of follow-up strategies. Icelandic residents born before 1976 were offered participation. Blood samples are collected alongside blood sampling in the Icelandic healthcare system. Participants with MGUS are randomized to three study arms. Arm 1 is not contacted, arm 2 follows current guidelines, and arm 3 follows a more intensive strategy. Participants who progress are offered early treatment. Samples are collected longitudinally from arms 2 and 3 for the study biobank. All participants repeatedly answer questionnaires on various exposures and outcomes including quality of life and psychiatric health. National registries on health are cross-linked to all participants. Of the 148,704 individuals in the target population, 80 759 (54.3%) provided informed consent for participation. With a very high participation rate, the data from the iStopMM study will answer important questions on MGUS, including potentials harms and benefits of screening. The study can lead to a paradigm shift in MM therapy towards screening and early therapy.

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Publisher Correction: Prevalence of monoclonal gammopathy of undetermined significance (MGUS) at HIV diagnosis in individuals 18–40 years old: a possible HIV indicator condition

Blood Cancer Journal ◽

10.1038/s41408-021-00501-8 ◽

2021 ◽

Vol 11 (6) ◽

Author(s):

Michele Bibas ◽

Silvia Pittalis ◽

Nicoletta Orchi ◽

Gabriella De Carli ◽

Chiara Agrati ◽

...

Keyword(s):

Monoclonal Gammopathy ◽

Hiv Diagnosis ◽

Undetermined Significance

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Overweight/Obesity and Monoclonal Gammopathy of Undetermined Significance

Clinical Lymphoma Myeloma & Leukemia ◽

10.1016/j.clml.2021.01.008 ◽

2021 ◽

Author(s):

Rebecca Georgakopoulou ◽

Angeliki Andrikopoulou ◽

Theodoros N. Sergentanis ◽

Oraianthi Fiste ◽

Flora Zagouri ◽

...

Keyword(s):

Monoclonal Gammopathy ◽

Undetermined Significance

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Serum BCMA levels predict outcomes in MGUS and smoldering myeloma patients

Blood Cancer Journal ◽

10.1038/s41408-021-00505-4 ◽

2021 ◽

Vol 11 (6) ◽

Author(s):

A. Visram ◽

C. Soof ◽

S. V. Rajkumar ◽

S. K. Kumar ◽

S. Bujarski ◽

...

Keyword(s):

Monoclonal Gammopathy ◽

Serum Samples ◽

Risk Models ◽

Independent Validation ◽

Increased Risk ◽

Risk Of Progression ◽

Paired Serum ◽

Smoldering Myeloma ◽

Undetermined Significance

AbstractSoluble BCMA (sBCMA) levels are elevated in monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM). However, the association between sBCMA levels and prognosis in MGUS and SMM has not been studied. We retrospectively analyzed sBCMA levels in stored samples from 99 MGUS and 184 SMM patients. Baseline sBCMA levels were significantly higher in MGUS and SMM patients progressing to MM during clinical follow up. When stratified according to the median baseline sBCMA level for each cohort, higher levels were associated with a shorter PFS for MGUS (HR 3.44 comparing sBCMA ≥77 vs <77 ng/mL [95% CI 2.07–5.73, p < 0.001] and SMM (HR 2.0 comparing sBCMA ≥128 vs <128 ng/mL, 95% 1.45–2.76, p < 0.001) patients. The effect of sBCMA on PFS was similar even after adjusting for the baseline MGUS or SMM risk stratification. We evaluated paired serum samples and found that sBCMA increased significantly in MGUS and SMM patients who eventually progressed to MM, whereas among MGUS non-progressors the sBCMA level remained stable. While our results require independent validation, they suggest that sBCMA may be a useful biomarker to identify MGUS and SMM patients at increased risk of progression to MM independent of the established risk models.

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