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Increased Frequency of Monoclonal Gammopathy of Undetermined Significance in Patients With Nonimmune Chronic Idiopathic Neutropenia Syndrome

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Abstract

This study describes the frequency of monoclonal gammopathy of undetermined significance (MGUS) and the changes in some inflammation-related serum proteins in 157 patients with nonimmune chronic idiopathic neutropenia syndrome (NI-CINS). Of these patients, 42 had pronounced neutropenia with neutrophil counts < 1500/μL, and 115 had mild neutropenia with neutrophil counts ranging from 1500 to 2499/μL. Sixty-six volunteers served as healthy control subjects and 157 ageand sex-matched patients hospitalized for nonmalignant diseases served as patient control subjects. We found that 28.6% of patients with pronounced neutropenia and 14.8% of patients with mild neutropenia had increased serum gamma globulins (above the 95% confidence limit of values of the control subjects). In the group of patients with pronounced neutropenia, 30.9% had increased immunoglobulin (Ig)G values and 23.8% had increased IgA values. In the group of patients with mild neutropenia, 17.4% had increased IgG values and 21.7% had increased IgA values. IgG and IgA values strongly correlated with the neutrophil count. No changes in serum IgM were found. Three of 42 patients with pronounced neutropenia (7.14%) and 3 of 115 patients with mild neutropenia (2.61%) had serum immunofixation tests which showed a small monoclonal spike—4 were IgG-K type, 1 was IgG-γ type, and 1 was IgA-K type. None of the healthy or patient control subjects had any evidence of MGUS. No significant changes in the amount of monoclonal spikes were documented during an 18- to 143-month follow-up (median, 58 months). Except for significantly increased α1-antitrypsin levels, there were no significant differences in the levels of acute-phase proteins studied between the study patients and the control subjects. These findings are consistent with our previous report suggesting the possible existence of an unrecognized low-grade chronic inflammation in patients with NI-CINS, which may be involved in the pathogenesis of neutropenia in the affected subjects.

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Authors and Affiliations

  1. Department of Hematology, University of Crete School of Medicine, University Hospital of Heraklion, Crete, Greece

    Helen A. Papadaki, Dimitris G. Eliopoulos, Charis Ponticoglou & George D. Eliopoulos

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  1. Helen A. Papadaki
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  2. Dimitris G. Eliopoulos
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  3. Charis Ponticoglou
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  4. George D. Eliopoulos
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Correspondence to George D. Eliopoulos.

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Papadaki, H.A., Eliopoulos, D.G., Ponticoglou, C. et al. Increased Frequency of Monoclonal Gammopathy of Undetermined Significance in Patients With Nonimmune Chronic Idiopathic Neutropenia Syndrome. Int J Hematol 73, 339–345 (2001). https://doi.org/10.1007/BF02981959

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  • DOI: https://doi.org/10.1007/BF02981959

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