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Clinical and pathology characteristics of 197 patients with monoclonal gammopathies of renal significance and paraprotein-related kidney damage in malignant lymphoproliferative disorders

https://doi.org/10.28996/2618-9801-2024-1-55-70

Abstract

Background. Paraprotein-related kidney damage in B-cell malignancies (BCM) often share common features with monoclonal gammopathy of renal significance (MGRS). In this single-center retrospective study, we aimed to analyze clinical presentation and pathology patterns of paraprotein-related kidney damage in malignant and non-malignant B-cell disorders. Materials and methods. Study group included 197 patients (M/F ratio 47.7/52.8%, mean age 59.41±10.44 years), with pathology-proven paraprotein-related kidney disease, followed in 1997-2022. For the further analysis, we split the patients into two sub-groups; MGRS sub-group consisted of 168 (85.2%) cases, and BCM sub-group included 29 (14.7%) cases. Results. In the MGRS sub-group general weakness, dyspnea, arterial hypotension, nephrotic syndrome and presence of monoclonal light chains lambda were found significantly more often compared to the BCM sub-group (p=0.011, p=0.009, p=0.010, p=0.011, and р=0.015 respectively). In the BCM sub-group significantly more often were arterial hypertension, presence of monoclonal light chains kappa and bone marrow plasmacytosis (p=0.048, р=0.003 and p<0.001 respectively). AL amyloidosis apparently dominated in the MGRS sub-group, constituting 72.6% of cases; in the BCM sub-group it was found in 31.0% of cases. Monoclonal immunoglobulin deposition disease was the more often finding in the BCM sub-group compared to MGRS (24.1% vs 11.3% respectively), however also yielding AL amyloidosis (24.1% vs 31.0% respectively). Cast-nephropathy, as well as light chain proximal tubulopathy, presented not exclusively in multiple myeloma, but also in the MGRS setting, but were both more than ten times often in BCM (13.7% vs 1.1% and 10.3% vs 0.5% respectively). Proliferative glomerulonephritis with monoclonal immunoglobulin deposits, atypical membranous nephropathy, cryoglobulinemic glomerulonephritis and C3 glomerulonephritis were found in MGRS sub-group only; and glomerulonephritis with antibodies to glomerular basement membrane. Non-proliferative glomerulonephritis with immunoglobulin M monoclonal deposits presented in the single cases in both sub-groups. Combination of two patterns of damage presented in BCM more than twice often, compared to MGRS (13.7% vs 5.3% respectively). Conclusions. AL amyloidosis, associated with monoclonal light chains lambda and presenting with general weakness, dyspnea, arterial hypotension and nephrotic syndrome is the most often pattern of paraprotein-related kidney damage, regardless malignant or non-malignant nature of the secreting clone; however, it is much more typical for MGRS than for BCM. Cast-nephropathy, traditionally regarded as a hallmark of multiple myeloma, also seen in smoldering myeloma, thus representing MGRS.

About the Authors

E. V. Zakharova
S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare; Russian Medical Academy of Continuing Professional Education of the Ministry of Health of the Russian Federation; A. I. Evdokimov Moscow State University of Medicine and Dentistry
Russian Federation


A. S. Zykova
S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare
Russian Federation


T. A. Makarova
S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare
Russian Federation


E. S. Leonova
S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare
Russian Federation


E. N. Nikitina
S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare
Russian Federation


E. S. Stolyarevich
A. I. Evdokimov Moscow State University of Medicine and Dentistry; City Clinical Hospital №52, Moscow City Department of Healthcare
Russian Federation


O. A. Vorobieva
National Center of Clinical Morphological Diagnostics
Russian Federation


E. V. Shutov
S.P. Botkin City Clinical Hospital, Moscow City Department of Healthcare; Russian Medical Academy of Continuing Professional Education of the Ministry of Health of the Russian Federation
Russian Federation


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Zakharova E.V., Zykova A.S., Makarova T.A., Leonova E.S., Nikitina E.N., Stolyarevich E.S., Vorobieva O.A., Shutov E.V. Clinical and pathology characteristics of 197 patients with monoclonal gammopathies of renal significance and paraprotein-related kidney damage in malignant lymphoproliferative disorders. Nephrology and Dialysis. 2024;26(1):55-70. (In Russ.) https://doi.org/10.28996/2618-9801-2024-1-55-70

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