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Fulminant type I cryoglobulinemic glomerulonephritis with unique ultrastructural plugs: a case report

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Abstract

Introduction: Type I cryoglobulinemia is a rare disease which affects the skin, central nervous system and kidneys. It is usually associated with lymphoproliferative disorders such as multiple myeloma, lymphoma and monoclonal gammopathy of renal significance. Proteinuria and membranoproliferative glomerulonephritis are the most common renal manifestations; Case presentation: Here we report the case of a female patient in her late 40 s who had proteinuria accompanied by Raynaud’s phenomenon, high blood and plasma viscosity, hearing loss, and cardiac and central nervous system involvement. Monoclonal immunoglobulin G-λ protein was detected and serum was positive for cryoglobulin. Renal biopsy revealed massive cryo-plugs with unique ultrastructural appearance in the glomerular and peritubular capillary lumina. Immunofluorescence showed predominant IgG3/λ deposition in cryo-plugs. As reported, the clinical manifestations of this patient resulted from cryoprecipitate and hyperviscosity syndrome; Conclusion: Cryoglobulinemia should be considered as a possible diagnosis in patients with Raynaud’s phenomenon, hyperviscosity syndrome and monoclonal immunoglobulin.

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Acknowledgements

This work was supported by grants from the National Key Research and Development Program of China (2016YFC1305402), Shanghai Municipal Key Clinical Specialty (shslczdzk 02502), National Natural Science Foundation of China (30871001) and Core Facility of Basic Medical Sciences, Shanghai Jiao Tong University School of Medicine.

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Authors and Affiliations

  1. Department of Nephrology, Institute of Nephrology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, 197 Ruijin Er Road, Shanghai, 200025, China

    Qinjie Weng, Jing Xu, Pingyan Shen, Tian Xu, Chenni Gao, Jingyuan Xie, Hong Ren & Xiaoxia Pan

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  1. Qinjie Weng
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  2. Jing Xu
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  3. Pingyan Shen
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  4. Tian Xu
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  5. Chenni Gao
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  6. Jingyuan Xie
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  7. Hong Ren
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  8. Xiaoxia Pan
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Contributions

QW, JX contributed to the research aims, acquisition of data, data analysis, data interpretation, in writing the manuscript and in drafting the article. XP designed the study and its concept, contributed to interpretation of the data and writing the manuscript. TX, HR, PS, JX, CG contributed to data analysis and interpretation of the data. All authors contributed to the drafting of the article, revising it critically and finally approving the version to be submitted.

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Correspondence to Xiaoxia Pan.

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Weng, Q., Xu, J., Shen, P. et al. Fulminant type I cryoglobulinemic glomerulonephritis with unique ultrastructural plugs: a case report. Int J Hematol 114, 620–625 (2021). https://doi.org/10.1007/s12185-021-03194-8

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  • DOI: https://doi.org/10.1007/s12185-021-03194-8

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