Crystalglobulin-associated nephropathy presenting as MGRS in a case of monoclonal B-cell lymphocytosis: a case report

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Abstract

Background

Crystalglobulin-associated nephropathy (CAN), a rare subtype of monoclonal gammopathy, usually associated with multiple myeloma and occasionally monoclonal gammopathy of uncertain significance (MGUS), is characterized by occluding monoclonal pseudothrombi within renal glomerular capillaries and/or interstitial arterioles. Ultrastructurally, these pseudothrombi are unique for having a crystalline substructure. We describe a case of an adult patient with monoclonal B-cell lymphocytosis (MBL) and acute renal failure whose kidney biopsy revealed a rare diagnosis of CAN.

Case presentation

A 63-year old male presented with a 2-month history of edema, arthralgia and malaise. He had acute kidney injury with hematoproteinuria on urine analysis. Serum and urine protein electrophoresis were both negative. A renal biopsy however revealed features of CAN. Organomegaly, bone pain and lymphadenopathy were absent. A repeat serum electrophoresis was positive for IgA kappa and a free light chain assay showed elevated free kappa light chains. Flow cytometry done subsequently revealed a diagnosis of MBL, chronic lymphocytic leukemia (CLL) type.

Conclusion

CAN in association with MBL/CLL has not been previously described in literature, and our case highlights yet another instance of monoclonal gammopathy of renal significance (MGRS) where a small B-cell clone resulted in extensive renal pathology without systemic manifestations.

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Most cited references 21

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Light Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era.

Michael B Stokes, Anthony Valeri, Leal C. Herlitz … (2016)

Light chain proximal tubulopathy (LCPT) is characterized by cytoplasmic inclusions of monoclonal LC within proximal tubular cells. The significance of crystalline versus noncrystalline LCPT and the effect of modern therapies are unknown. We reported the clinical-pathologic features of 40 crystalline and six noncrystalline LCPT patients diagnosed between 2000 and 2014. All crystalline LCPTs were κ-restricted and displayed acute tubular injury. One-third of noncrystalline LCPT patients displayed λ-restriction or acute tubular injury. Only crystalline LCPT frequently required antigen retrieval to demonstrate monoclonal LC by immunofluorescence. In five of 38 patients, crystals were not detectable by light microscopy, but they were visible by electron microscopy. Hematolymphoid neoplasms, known before biopsy in only 15% of patients, included 21 monoclonal gammopathies of renal significance; 15 multiple myelomas; seven smoldering multiple myelomas; and three other neoplasms. Biopsy indications included Fanconi syndrome (38%; all with crystalline LCPT), renal insufficiency (83%), and proteinuria (98%). Follow-up was available for 30 (75%) patients with crystalline LCPT and all six patients with noncrystalline LCPT, of whom 11 underwent stem cell transplant, 16 received chemotherapy only, and nine were untreated. Complete or very good partial hematologic remissions occurred in six of 22 treated crystalline LCPT patients. By multivariable analysis, the only independent predictor of final eGFR was initial eGFR, highlighting the importance of early detection. All patients with crystalline LCPT treated with stem cell transplant had stable or improved kidney function, indicating the effectiveness of aggressive therapy in selected patients.

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Thrombotic microangiopathy associated with monoclonal gammopathy

Sanjeev Sethi, Ronald Go, Aishwarya Ravindran … (2017)

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Renal complications in chronic lymphocytic leukemia and monoclonal B-cell lymphocytosis: the Mayo Clinic experience.

Paolo Strati, Samih Nasr, Nelson Leung … (2015)

While the renal complications of plasma cell dyscrasia have been well-described, most information in patients with chronic lymphocytic leukemia and monoclonal B-cell lymphocytosis is derived from case reports. This is a retrospective analysis of patients with chronic lymphocytic leukemia or monoclonal B-cell lymphocytosis who underwent kidney biopsy for renal insufficiency and/or nephrotic syndrome. Between January 1995 and June 2014, 49 of 4,024 (1.2%) patients with chronic lymphocytic leukemia (n=44) or monoclonal B-cell lymphocytosis (n=5) had a renal biopsy: 34 (69%) for renal insufficiency and 15 (31%) for nephrotic syndrome. The most common findings on biopsy were: membranoproliferative glomerulonephritis (n=10, 20%), chronic lymphocytic leukemia interstitial infiltration as primary etiology (n=6, 12%), thrombotic microangiopathy (n=6, 12%), and minimal change disease (n=5, 10%). All five membranoproliferative glomerulonephritis patients treated with rituximab, cyclophosphamide and prednisone-based regimens had recovery of renal function compared to 0/3 patients treated with rituximab with or without steroids. Chronic lymphocytic leukemia infiltration as the primary cause of renal abnormalities was typically observed in relapsed/refractory patients (4/6). Thrombotic microangiopathy primarily occurred as a treatment-related toxicity of pentostatin (4/6 cases), and resolved with drug discontinuation. All cases of minimal change disease resolved with immunosuppressive agents only. Renal biopsy plays an important role in the management of patients with chronic lymphocytic leukemia or monoclonal B-cell lymphocytosis who develop renal failure and/or nephrotic syndrome.

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Author and article information

Contributors

Rajib K. Gupta:

ORCID: http://orcid.org/0000-0003-2499-9528

rajibgupta2011@gmail.com

Lois J. Arend: ljarend@jhu.edu

Anupama BK: BkA@upstate.edu

Sriram Narsipur: narsipur@upstate.edu

Ramya Bhargava: bhargavr@upstate.edu

Journal

Journal ID (nlm-ta): BMC Nephrol

Journal ID (iso-abbrev): BMC Nephrol

Title: BMC Nephrology

Publisher: BioMed Central (London )

ISSN (Electronic): 1471-2369

Publication date (Electronic): 18 May 2020

Publication date PMC-release: 18 May 2020

Publication date Collection: 2020

Volume: 21

Electronic Location Identifier: 184

Affiliations

[1 ]GRID grid.411023.5, ISNI 0000 0000 9159 4457, Department of Pathology, , SUNY Upstate Medical University, ; 750 E Adams Street, Syracuse, NY 13210 USA

[2 ]GRID grid.21107.35, ISNI 0000 0001 2171 9311, Department of Pathology, , Johns Hopkins University School of Medicine, ; Baltimore, MD USA

[3 ]GRID grid.411023.5, ISNI 0000 0000 9159 4457, Department of Medicine, , SUNY Upstate Medical University, ; Syracuse, NY USA

[4 ]GRID grid.411023.5, ISNI 0000 0000 9159 4457, Department of Medicine and Nephrology, , SUNY Upstate Medical University, ; Syracuse, NY USA

[5 ]GRID grid.411023.5, ISNI 0000 0000 9159 4457, Department of Nephrology, , SUNY Upstate Medical University, ; Syracuse, NY USA

Author information

Rajib K. Gupta http://orcid.org/0000-0003-2499-9528

Article

Publisher ID: 1818

DOI: 10.1186/s12882-020-01818-5

PMC ID: 7236346

PubMed ID: 32423442

SO-VID: 80c625fa-a199-4e1a-95b5-193c30dfcc4c

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

History

Date received : 1 January 2020

Date accepted : 19 April 2020

Custom metadata

ScienceOpen disciplines: Nephrology

Keywords: crystalglobulin-associated nephropathy,monoclonal b-cell lymphocytosis,monoclonal gammopathy of renal significance

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ScienceOpen disciplines: Nephrology

Keywords: crystalglobulin-associated nephropathy, monoclonal b-cell lymphocytosis, monoclonal gammopathy of renal significance

Crystalglobulin-associated nephropathy presenting as MGRS in a case of monoclonal B-cell lymphocytosis: a case report

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Abstract

Background

Case presentation

Conclusion

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Karger: Nephrology

Most cited references 21

Light Chain Proximal Tubulopathy: Clinical and Pathologic Characteristics in the Modern Treatment Era.

Thrombotic microangiopathy associated with monoclonal gammopathy

Renal complications in chronic lymphocytic leukemia and monoclonal B-cell lymphocytosis: the Mayo Clinic experience.

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