P0257CLINICOPATHOLOGICAL SPECTRUM OF KIDNEY INVOLVEMENT IN MONOCLONAL GAMMOPATGY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Hadj Brahim Mayssa ◽  
Meriem Ben salem ◽  
Ouni Amal ◽  
Handous Insaf ◽  
Bensalah Manel ◽  
...  
Keyword(s):  
Renal Failure ◽  
Nephrotic Syndrome ◽  
Serum Proteins ◽  
Kidney Diseases ◽  
Renal Involvement ◽  
Chronic Hemodialysis ◽  
Al Amyloidosis ◽  
Monoclonal Gammopathies ◽  
Cast Nephropathy ◽  
Kidney Involvement

Abstract Background and Aims The occurrence of kidney diseases associated with a monoclonal gammopathy is increasingly recognized.Renal biopsy and evaluation by light microscopy, electron microscopy and immunofluorescence,in association with adequate clinical examination are essential in accurate diagnosis. The objective of our study is to describe the types of kidney damage secodary to monoclonal gammopathies and to study its renal prognosis. Method It is a monocentric RETOSPECTIVE DESCRIPTIVE STUDY carried out in the nephrology department of CHU FATTOUMA BOURGUIBA MONASTIR spread over a period of 7 years from January 2012 to December 2018. Results we collected 40 cases of monoclonal gammopathies. The median age was 61 years.The sex ratio was 0,9. the circumstances of discovery were dominated by renal failure. it revealed the diagnosis in 77.5% of cases. the renal presentation proteinuria in 70%, microscopic hematuria in 12.5%, edematous syndrome in 27.5% and nephrotic syndrome in 37,5%. The mean creatinine level was 500 umol / L and proteinuria at 4,3 g / 24 h with a maximum rate of 17.5 g / 24h. the electrophoresis of serum proteins showed the presence of a monoclonal peak in 90% of the cases in the gamma zone. The plasmocytosis was less than 10% in 15% of cases and more than 60% in 22,5% of the cases. Radiology showed bone localizations in 32,5% of cases. These gammopathies were classified as Multiple myeloma in34 patients, POEMS in one case and 5 MGRS. Kidney biopsy was performed in 10 patients showing cast nephropathy, AL amyloidosis and vasculites(endocapillary glomerulonephritis with hyaline thrombi in all vessels and double contour appearance of the glomerular basement membrane) in respectively 12,5%, 7,5% and 2,5% of cases. the main indications were rapidly progressive renal failure, unexplained acute renal failure and nephrotic syndrome. The renal involvement retained was a myeloma tubulopathy in 28 patients, 3 of whom were confirmed histologically . AL amyloidosis was found in 5 patients and the confirmation was histological. Type 1 cryoglobulinemia was diagnosed in only one patient. Glomerular nephropathy remained undetermined in 6 patients whose PBR was indicated but not made in the presence of hemostasis disorders. The chemotherapy was started in 34 patients based on bortezomide-thalidomide-dexamethasone in 30% of cases. An autograft of the bone marrow was done in one patient. Hemodialysis was required in 15 patients. The renal function was improved in 42.5% of the cases and a passage on chronic hemodialysis in 10% of cases. 10% of patients have achieved complete hematological remission. Conclusion Patients with MG can develop a large variety of related renal lesions.Renal involvement darkens the prognosis hence the need for adequate care .

scholarly journals Clinical Presentation and Renal Histopathological Findings in Patients with Monoclonal Gammopathy and Kidney Disease

2020 ◽  
Author(s):  
Gaetano Alfano ◽  
Alice Delrio ◽  
Francesco Fontana ◽  
Annachiara Ferrari ◽  
Giacomo Mori ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Monoclonal Gammopathy ◽  
Renal Involvement ◽  
Lymphoproliferative Disorders ◽  
M Protein ◽  
Al Amyloidosis ◽  
Light Chain Deposition Disease ◽  
Monoclonal Gammopathies ◽  
Increased Risk ◽  
Kidney Involvement

AbstractMonoclonal gammopathies have been widely associated with renal lesions. Nephrotoxicity of the secreted monoclonal (M)-protein relies on a complex interplay between biological characteristics and serum concentration. Little is known about the prevalence and renal manifestations of the different types of monoclonal gammopathies in patients with kidney disease.We reviewed all renal biopsies in our Center during a 12-year period to characterize patients diagnosed with monoclonal gammopathy. Data about demographics, laboratory examinations, renal manifestations and histological lesions were collected retrospectively. Results were correlated with the different lymphoproliferative disorders to evaluate the relationship between renal involvement and monoclonal gammopathies.Monoclonal gammopathy was detected in 179 (13.4%) patients. The circulating M-protein was secreted by monoclonal gammopathy of undetermined significate (MGUS) (51.9%), myeloma multiple (MM) (25.7%), primary amyloidosis (AL) (8.9%), smoldering MM (5 %), non-Hodgkin lymphoma (NHL) (6.7%) and HL (1.7%). Documented renal involvement in benign disorders such as MGUS and SMM accounted for 7.5% and 11.1%, respectively. MM was associated with an increased risk of kidney involvement (adjusted odds ratio=36.4; P=<0.001) and manifested with higher serum creatinine compared to other disorders. AL amyloidosis was principally secondary to MGUS (75%) and presented with nephrotic proteinuria. NHL and HL patients had heterogeneous renal manifestations. MGRS manifested both with light chain deposition disease and membranoproliferative glomerulonephritis. Compared to the other lymphoproliferative disorders, MM and AL amyloidosis showed higher creatinine blood levels and proteinuria, respectively. MM was significantly associated with kidney disease in our cohort of patients.Monoclonal gammopathy is a frequent diagnosis in patients with kidney disease. An accurate diagnostic process including lab tests and kidney biopsy is necessary to identify if the secreted M-protein is associated with renal involvement.

scholarly journals Renal involvement as the first symptom of multiple myeloma

2019 ◽  
Author(s):  
Yongjing Du ◽  
Ping Zhang ◽  
Xiang Zhong ◽  
Shasha Chen ◽  
Guisen Li ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Nephrotic Syndrome ◽  
Light Chain ◽  
Clinical Laboratory ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Renal Amyloidosis ◽  
Cast Nephropathy ◽  
Immunofixation Electrophoresis

Abstract Background . Renal involvement is a common complication of multiple myeloma (MM). However, most studies have focused on renal failure in MM, and little information is available about the other renal manifestations in MM and their association with immunophenotypes and renal pathology. Methods . We retrospectively analyzed the clinical, laboratory and pathology data of 283 MM patients treated in Sichuan Provincial People’s Hospital, West China, between January 1990 and May 2017. The patients were divided into a renal involvement group (n = 200) and a non-renal involvement group (n = 83). Results. In the renal involvement group, 90 (45.0%) patients were diagnosed with MM in the Nephrology department, and isolated proteinuria, renal failure and nephrotic syndrome were detected in 90(45.0%), 94 (47.0%) and 58 (29.0%) patients, respectively. 135 patients with renal involvement underwent immunofixation electrophoresis, and IgG, IgA, IgD, IgE, pure light chain and nonsecretory MM were detected in 52 (38.5%), 32 (23.7%), 1 (0.7%), 1 (0.7%), 45(33.3%) and 4 (3.0%) patients, respectively. 47 patients without renal involvement also underwent immunofixation electrophoresis, and IgG and IgA MM were found in 24 (51.0%) and 18 (38.3%) patients, respectively. Severe anemia and hypertension, hypercalcemia and pure light chain were more frequent in patients with renal involvement (P < 0.05).9 patients with renal involvement were performed renal biopsy, and cast nephropathy, renal amyloidosis were proved in 5 and 4 patients, respectively. Conclusions. Renal involvement was common at MM diagnosis and had diverse clinical manifestations. The most common clinical manifestations include renal failure, isolated albuminuria and nephrotic syndrome. Nephrologists should rule out MM in patients presenting with renal involvement.

DETERMINING OF MONOCLONAL GAMMOPATHY IN NEPHROLOGICAL PATIENTS

2019 ◽  
Vol 23 (2) ◽  
pp. 82-90
Author(s):  
L. B. Lysenko ◽  
N. V. Chebotareva ◽  
N. N. Mrykhin ◽  
V. V. Rameev ◽  
T. V. Androsova ◽  
...  
Keyword(s):  
Renal Diseases ◽  
Chronic Glomerulonephritis ◽  
Al Amyloidosis ◽  
Hematological Neoplasms ◽  
Cast Nephropathy ◽  
Number Of Patients ◽  
Light Chain Disease ◽  
Kidney Involvement ◽  
Cryoglobulinemic Glomerulonephritis ◽  
Electrophoresis Of Proteins

BACKGROUND. Мonoclonal gammopathy (MG) is not only the state preceding of hematological neoplasms, but also associated with non- hematological diseases, in particular damage of kidneys. Earlier diagnosis of MG represents an important area in treating patients with renal diseases associated with MG. THE AIM: To determine the frequency of MG among therapeutic and nephrological patients for optimization of methods of their diagnosis and treatment. PATIENTS AND METHODS: In common, 11392 patients were analyzed within 4 years (2013-2016). The standard clinical examination was conducted. Method of an electrophoresis of proteins of serum of blood and the 24-hour urine, method of immunofixation of proteins of serum and urine, and method of free light chains definition in serum (Freelite) were used for MG identification. RESULTS: MG is diagnosed in 174 of 11392 patients: 49 % of men and 51 % of women aged from 18 up to 85 years. MG was found 2.1 times more often in nephrological patient than in patients of therapeutic departments. Among patients of this group, AL-amyloidosis with kidney involvement was diagnosed in 41 %, cryoglobulinemic glomerulonephritis – in 18 %, chronic glomerulonephritis – in 35 %, also there was small number of patients with light chain disease and cast-nephropathy. 86 % of nephrological patients had less than 5 g/l of monoclonal protein that corresponds oligo secretory MG, and at 46 % from them – less than 1 g/l, other 10 % had MG of 5-10 g/l, and only in 4.42 % of patients MG more 10g/l was defined. CONCLUSION: We conclude that MG, especially oligo secretory form, play a significant role in pathogenesis of renal damage. It is important to apply sensitive methods – immunofixation of proteins and method «Freelite» for nephrological patients.

P0498CLINICAL PRESENTATION AND RENAL HISTOPATHOLOGICAL FINDINGS IN PATIENTS WITH MONOCLONAL GAMMOPATHY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Gaetano Alfano ◽  
Alice Delrio ◽  
Francesco Fontana ◽  
Annachiara Ferrari ◽  
Andrea Solazzo ◽  
...  
Keyword(s):  
Renal Function ◽  
Monoclonal Gammopathy ◽  
Kidney Biopsy ◽  
Renal Involvement ◽  
M Protein ◽  
Histological Evaluation ◽  
Al Amyloidosis ◽  
Creatinine Ratio ◽  
Urine Protein ◽  
Cast Nephropathy

Abstract Background and Aims Monoclonal gammopathy is associated with renal lesions due to the toxic effect of M-protein. The aim of our study was to evaluate the prevalence and the clinical presentation of monoclonal gammopathy in patients who underwent kidney biopsy for renal impairment. Method We conducted a retrospective study at the Nephrology and Dialysis Unit of the University Hospital of Modena from 2005 to 2017. The diagnosis of renal disease was proved histologically. Results Monoclonal gammopathy was found in 179 out of 1334 patients (13.4%). Mean age was 66.1±13.4 years with a predominance of males (63.7%). There was no differences (P=0.16) between the age of patients with benign (64.9±14.3) and malignant lymphoproliferative diseases (67.6±12). The hematologic disorders involved in the production of M-protein were MGUS (51.9%), myeloma multiple (MM) (25.7%), amyloidosis (8.9%) smoldering MM (5 %), non-Hodgkin lymphoma (NHL) (6.7%) and HL (1.7%). The prevalence of MGUS was estimated to be 6.97% (93/1334). Mean serum creatinine was 2.68±2.12 mg/dl (eGFR of 35.24±29.32 ml/min) and urine protein/creatinine ratio of 5.1±6.5. None of the study subjects progressed to MM or other lymphoproliferative diseases. The most common kidney disease was membranoproliferative (GN) (19.3%). MGRS has been identified in five patients (5.4%). Mean age of MM was 66.84±13 years. Serum concentration M-protein was 1.47±0.98. Among patients with AKI (89.1%), 13 patients (28.2%) required urgent hemodialysis. Histological evaluation showed cast nephropathy (71.7%), MM-associated AL amyloidosis (15.2%), MM-associated-light chain deposition disease (4.3%) and interstitial nephritis (8.7%). Nine patients had a diagnosis of smoldering MM. Average age was 69.17±10.8 years old. At presentation, creatinine was 2.31±2.6 mg/dl (GFR of 41.35 ml/min). Evaluation of renal biopsies allowed us to identify different patterns of glomerular diseases, expression of an aspecific renal involvement of this hematological disease. AL amyloidosis was secondary to MGUS (75%) and smoldering MM (33%). Mean age was 66.04±11.7 years old. At presentation mean urine protein/creatinine ratio was 8.33±3.2 concomitant to a serum albumin level of 2.74±0.84 gr/dl. Mean creatinine was 1.4 mg/dl corresponding to eGFR= 56.5 ml/min. Average age of NHL patients was 72.6±9.6 years. Renal function was extremely variable at presentation; mean creatinine was 2.4±1.6 mg/dl (eGFR of 30.4±22.7 ml/min). Histological evaluation of biopsy specimen revealed amyloidosis (25%), cryoglobulinemic GN (25%), LCDD (16.6%), cast-nephropathy (8.3%), LCDD (8.3%) and other renal diseases (16.8%). Three patients (1.12%) had a diagnosis of HL at mean age of 69.04±5.3 years. At presentation, renal function was normal in all patients with a creatinine of 0.93±0.07mg/dl (eGFR of 62.7.3±7.4 ml/min). Urine protein/creatinine ratio was 0.3±0.2. Kidney biopsy revealed cryoglobulinemic GN (75%) and hypertensive nephrosclerosis (25%). ANOVA analysis did not found statistically significant differences in age (p=0.11) and serum concentration of M-protein (P=0.42) between groups. The differences in mean serum creatinine and mean urine protein/creatinine ratio were statistically significant between groups, (P&lt;0.0001) and (P=0.003), respectively. A post hoc Tukey test showed that proteinuria was higher in AL amyloidosis compared to MM and HL, whereas renal function was worse in MM patients compared to the others. Conclusion MGUS was the most common monoclonal gammopathy. Surprisingly, it is frequently associated with membranoproliferative glomerulonephritis. MGRS is a rare histopathological entity (5.4%). MM manifests frequently with AKI whereas AL amyloidosis with nephrotic syndrome. Renal function was extremely variable in NHL patients; on the other hand, the limited number of HL patients with renal involvement in our cohort does not allow generalization of our findings.

scholarly journals Renal involvement as the first symptom of multiple myeloma

2019 ◽  
Author(s):  
Yongjing Du ◽  
Ping Zhang ◽  
Xiang Zhong ◽  
Shasha Chen ◽  
Guisen Li ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Light Chain ◽  
Clinical Laboratory ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Renal Amyloidosis ◽  
Cast Nephropathy ◽  
Light Chain Disease ◽  
Immunofixation Electrophoresis

Abstract Background Renal involvement is a common complication of multiple myeloma (MM). However, most studies have focused on renal failure in MM, and little information is available about the other renal manifestations in MM and their association with immunophenotypes and renal pathology. Methods We retrospectively analyzed the clinical, laboratory and pathology data of 283 MM patients treated in Sichuan Provincial People’s Hospital, West China, between January 1990 and May 2017. The patients were divided into a renal involvement group (n = 200) and a non-renal involvement group (n = 83). Results In the renal involvement group, 90 (45.0%) patients were diagnosed with MM in the Nephrology department, and isolated proteinuria, renal failure and nephrotic syndrome were detected in 90(45.0%), 94 (47.0%) and 53 (27.0%) patients, respectively. 135 patients with renal involvement underwent immunofixation electrophoresis, and IgG, IgA, IgD, IgE, pure light chain and nonsecretory MM were detected in 52 (38.5%), 32 (23.7%), 1 (0.7%), 1 (0.7%), 45(33.3%) and 4 (3.0%) patients, respectively. 47 patients without renal involvement also underwent immunofixation electrophoresis, and IgG and IgA MM were found in 24 (51.0%) and 18 (38.3%) patients, respectively. Severe anemia and hypertension, hypercalcemia and pure light chain disease were more frequent in patients with renal involvement (P < 0.05). 9 patients with renal involvement were performed renal biopsy, and cast nephropathy and renal amyloidosis were proved in 5(55.6%) and 4(44.4%) patients, respectively. Conclusions Renal involvement was common at MM diagnosis and had diverse clinical manifestations. Nephrologists should rule out MM in patients presenting with renal involvement.

scholarly journals MO196KIDNEY INVOLVEMENT IN WALDENSTROM MACROGLOBULINEMIA AND IGM MONOCLONAL GAMMOPATHY

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Sara Cardoso Fernandes ◽  
Ricardo Gomes ◽  
Ana Messias ◽  
Bernardo Do Sacramento Marques Da Costa ◽  
Mário Góis ◽  
...  
Keyword(s):  
Renal Disease ◽  
Kidney Diseases ◽  
Wide Spectrum ◽  
Laboratory Data ◽  
Al Amyloidosis ◽  
Waldenström Macroglobulinemia ◽  
Hematologic Disorder ◽  
Waldenstrom Macroglobulinemia ◽  
Kidney Involvement ◽  
Renal Histology

Abstract Background and Aims Renal manifestations of IgM gammopathy are less common than those seen in patients with multiple myeloma. However, a wide spectrum of kidney diseases has been described in previous publications. We aim to characterize kidney involvement in patients with Waldenström Macroglobulinemia and IgM-secreting B cell lymphoproliferative disorders. Method We retrospectively studied 7 patients with a circulating monoclonal IgM and renal histology showing evidence of monoclonal immunoglobulin deposits or lymphomatous infiltration. Demographic, clinical and laboratory data were collected. Results Of the 7 patients studied, four (57%) were male and the median age was 68 years old (range 41-79). Among the 7 patients, 5 met criteria for Waldenström Macroglobulinemia after bone marrow biopsy; none of them had hyperviscosity syndrome. Four patients presented with nephrotic syndrome and all had impaired renal function and hypertension. Four patients had microscopic hematuria and only one patient showed no proteinuria. Mean serum creatinine levels were 2.9mg/dL. Renal biopsy showed different patterns of renal injury, including typical intracapillary monoclonal deposits disease (5 patients), membranoproliferative glomerulonephritis with cryoglobulinemia (1 patient), AL-amyloidosis (1 patient) and interstitial lymphoplasmacytic infiltration with CD20+ lymphocytes associated with minimal change disease. Follow-up data were obtained in 4 patients: 3 underwent chemotherapy but only one achieved complete remission and 2 progressed to end-stage renal disease. Conclusion Although rare, Waldenström Macroglobulinemia and IgM gammopathy are responsible for diverse manifestations of renal disease. A prompt diagnosis is of utmost importance in order to ensure early start of therapy, which should be directed at the underlying hematologic disorder, to improve renal survival.

P0457MALE LUPUS NEPHRITES: WHAT ABOUT RISK FACTORS FOR RENAL FAILURE ? EXPERIENCE OF ONE DEPARTMENT

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Kharroubi Melek ◽  
Raja Trabelsi ◽  
Mondher Ounissi ◽  
Soumaya Chargui ◽  
Mouna Jerbi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Renal Failure ◽  
Nephrotic Syndrome ◽  
Lupus Nephritis ◽  
Activity Index ◽  
Renal Involvement ◽  
Pathological Finding ◽  
Rank Test ◽  
Renal Prognosis ◽  
Male Patients

Abstract Background and Aims The occurrence of renal involvement during the clinical course of systemic lupus erythematous (SLE) is generally considered to be the most important factor influencing the prognosis in terms of morbidity and mortality. The factors influencing prognosis in lupus nephritis (LN) are variable in literature. The aim of our study was to analyze the clinicopathological correlations, and risk factors associated with renal failure in male patients with LN. Method We retrospectively studied all male patients with kidney biopsy-proven lupus nephritis (LN) treated in our department during the period between 1979 and 2016. Looking for predictive factors related to renal prognosis, we analyzed clinical, biological and histological data by multivariate analysis using the comparison of the survival rates by the log-rank test. Results We collected 41 native renal biopsies showing LN. Patients were aged 32.17 years (17-65 years) at the time of diagnosis of LN. Diagnosis of SLE was made according to the criteria of the American College of Rheumatology. The most common clinical presentation was nephrotic syndrome (61%), and the most frequent pathological finding in sediment was proteinuria (85%). At the time of diagnosis, 17% of patients had hypertension and 39 % of patients had an eGFR under than 60 ml/min/1.73 m2. LN was of class I, II, III, IV and V in 7.5%, 12.5%, 27.5%, 47.5% and 41.5% of the cases, respectively. Fourteen patients developed end-stage renal failure. The presence of hematuria, renal failure at the time of diagnosis, nephrotic syndrome, low complement, proliferative glomerulonephritis (class IV), high activity index score of LN and thrombotic microangiopathy were significantly associated with poor renal prognosis with (p=0.0053), (p=0.0002), (p=0.0186), (p=0.0287), (p=0.0005), (p=0.058), (p=0.0117), respectively. Conclusion Renal failure at the time of diagnosis and active proliferative lesions should be diagnosed and treated as soon as possible because they influence the renal prognosis in lupus man.

scholarly journals Renal Amyloidosis: A Synopsis of its Clinical Presentation, Diagnosis and Treatment

2015 ◽  
Vol 1 (1) ◽  
pp. napoc.2015.1460
Author(s):  
Elena V. Zakharova
Keyword(s):  
Renal Failure ◽  
Amyloid Fibrils ◽  
Renal Involvement ◽  
Pharmacological Therapy ◽  
Systemic Amyloidosis ◽  
Renal Amyloidosis ◽  
High Dose ◽  
Al Amyloidosis ◽  
Intracellular Space ◽  
High Dose Melphalan

Amyloidosis is a heterogeneous group of hereditary and acquired diseases in which normally soluble plasma proteins are deposited in the extracellular and/or intracellular space in abnormal, insoluble, fibrillar form. Renal damage is one of the most common features of systemic amyloidosis, and the presentation is most commonly due to the consequences of renal involvement, with proteinuria and progressive renal decline. Progression to end-stage renal failure is common. Early diagnosis of systemic amyloidosis is difficult. Renal amyloidosis typically presents with nephrotic syndrome and/or renal failure. Treatment of AL amyloidosis aims to reduce production of the monoclonal immunoglobulin precursorvia chemotherapy. Current options for treatment include melphalan+dexamethasone or cyclophosphamide-bortezomib-dexamethasone regimens, or in selected patients, high-dose melphalan with autologous stem cell transplantation. The focus of current research is on pharmacological therapy to solubilize amyloid fibrils and increase tissue catabolism of amyloid deposits.

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