scholarly journals P0463KIDNEY INVOLVEMENT IN PARAPROTEINEMIC DISEASES - ANALYSIS OF THE SINGLE CENTER REGISTER OF KIDNEY BIOPSIES

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Nikola Zagorec ◽  
Ana Šavuk ◽  
Ivica Horvatić ◽  
Dino Kasumović ◽  
Ana Brechelmacher ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Plasma Cell ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Kidney Biopsy ◽  
Kidney Injury ◽  
Al Amyloidosis ◽  
Monoclonal Immunoglobulin

Abstract Background and Aims Kidneys are often damaged in paraproteinemic conditions. Paraproteins are monoclonal immunoglobulins or immunoglobulin fractions that are produced by a clonal population of B- or plasma cell lineage and can cause a variety of histological patterns of kidney injury, such as light chain (AL) amyloidosis or light chain cast nephropathy (LCCN). Monoclonal gammopathy of renal significance (MGRS) represents a group of disorders in which a monoclonal immunoglobulin secreted by B- or plasma cell clone causes renal damage. By definition, these disorders do not meet diagnostic criteria for overt, symptomatic multiple myeloma or a lymphoproliferative disorder, but in contrast to monoclonal gammopathy of undetermined significance (MGUS) there is evidence of end-organ damage that can warrant therapy. Method All patients with paraproteinemic kidney disease were identified by retrospective review of the Hospital Register of kidney biopsies done at Department of Nephrology and Dialysis, in Dubrava University Hospital, Zagreb, from 2009 until 2018. Every kidney biopsy was analyzed by light, immunofluorescent and electron microscopy. Laboratory findings, including serum protein electrophoresis, serum free light chain level and immunofixation of serum proteins, were done for every patient. Clinical and histologic features of patients and features of underlying hematological conditions were analyzed. Results We identified 47 patients (3,28% of all biopsies that were done in that period) with kidney disease with clear hematological background. The mean patients' age at the time of the biopsy was 63 years and 27 of them were females. Two patients had signs of direct infiltration of kidneys with malignant lymphomic cells (non-Hodgkin lymphoma) and were excluded from the analysis. Clinical presentation of the patients at the time of biopsy were: proteinuria in 85% of patients, full nephrotic syndrome in 55%, azotemia in 66% of patients (80% had acute kidney injury of unclear etiology) and hematuria in 12,7%. Most common histologic patterns of kidney injury were AL amyloidosis (45%) and LCCN (30%) but additionally 7 different histological patterns were found: light chain depostion disease, light chain proxymal tubulopathy, fibrillary and imunotactoid glomerulopathy, proliferative glomerulonephritis with monoclonal immunoglobulin deposition, crioglobulinemic glomerulopathy type I and tubulointerstitial damage caused by immunoglobulin deposition. Figure 1 shows main features of patients with AL amyloidosis and cast LCCN. Conclusion Kidney disease can be initial presentation of an underlying paraproteinemia and, as our data showed, can clinicaly present with acute kidney injury, nephrotic or subnephrotic range proteinuria or full nephrotic syndrome. Variety of histologic patterns of kidney injury were described and AL amyloidosis and LCCN were the most common histological findings. Detailed hematologic workup should follow kidney biopsy in order to determine the exact nature and extension of the disease and therefore the most appropriate therapy.

scholarly journals P0204PREVALENCE AND PREDICTIVE FACTORS OF BIOPSY-PROVEN NEPHROPATHY RELATED TO MONOCLONAL GAMMOPATHY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Arthur Roux ◽  
Helene Lazareth ◽  
Dominique Nochy ◽  
Anne-Sophie Jannot ◽  
Camille Nevoret ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Serum Creatinine ◽  
Monoclonal Gammopathy ◽  
Kidney Biopsy ◽  
Hematological Malignancy ◽  
Al Amyloidosis ◽  
Monoclonal Immunoglobulin ◽  
Lymphoid Leukemia ◽  
Predictive Values ◽  
Bence Jones Proteinuria

Abstract Background and Aims The prevalence of monoclonal gammopathy (MG) and kidney disease increases with age. When a patient present with both conditions, it is often necessary to perform a kidney biopsy in order to rule out a Monoclonal Immunoglobulin-Related Nephropathy (MIRN) that may require a specific therapy that targets either an overt hematological malignancy or a MG or Renal Significance (MGRS). The aim of our study was to identify factors that predict the presence of MIRN in this setting. Method This retrospective monocentric study included all patients who underwent a kidney biopsy between 2007 and 2018 with concurrent presence of GM, as defined by positive serum immuno-electrophoresis and/or Bence-Jones proteinuria. Results 328 patients were included, representing 11.8% of all kidney biopsies performed in our center during this period. Indication of biopsy was renal failure (eGFR <60ml/min/1.73m) in 77.4% of cases and/or proteinuria (urine protein-to-creatinine ratio >0.5g/g) in 75.9% of them. Median (IQR) serum creatinine was 155 μmol/L (111-233), eGFR 37.5 (22-56) ml/min/1.73m, serum albumin 29.5 (24-35) g/l. Median age was 67 (57/75), the M/F ratio was 198/130, diabetes mellitus was present in 21.3% of cases, hypertension in 53.1%. Kidney biopsy revealed that nephropathy was related to MG in 91/328 patients (27.7%). Myeloma cast nephropathy and AL amyloidosis were the most common histopathological subtypes (36 and 34% respectively), followed by monoclonal immunoglobulin deposition disease (15%) and cryoglobulinemic glomerulonephritis (8%). Patients with MIRN had more severe renal function impairment with median (IQR) serum creatinine of 176 (119-307) vs 149 (108-216) μmol/l (p=0.003) and heavier proteinuria, 3.9 (2.2-8.2) vs 2.0 (0.9-5.2) g/g (p< 0.001), when compared to non-MIRN patients. Hematological malignancy was diagnosed in 83 cases (25,3%) (Multiple Myeloma in 62, non-Hodgkin Lymphoma in 6, Waldenström Macroglobulinemia in 6, Chronic Lymphoid Leukemia in 6, Plasmocytoma in 3). Among them, MIRN was diagnosed in 51 (61%) cases but tumoral lympho-plasmocytic infiltration was observed in 9 (11%) cases. In this subgroup of patients, no laboratory test could predict the presence of a specific nephropathy. Among patients with no hematological malignancy (n=245), MIRN was diagnosed in 40 cases (16%) to confirm MGRS diagnosis. The markers that were most commonly associated with the presence of MGRS were positive Bence-Jones proteinuria (OR 4.7; 95%CI 2.2-10.3; p< 0.001), abnormal serum Free Light Chain (FLC) ratio (OR 4.2; 95%CI 1.7-10.7; p< 0.001), and serum electrophoresis spike >1.5 g/l (OR 5.9; 95%CI 2.6-13.5; p< 0.001). However, none of those markers had sufficient power to formally predict the result of the biopsy, as positive (PPV) or negative (NPV) predictive values were 36/41/45 % and 89/86/88 % respectively. Conclusion Almost one-third of patients with MG and kidney disease referred to our Department have biopsy-proven related nephropathy. Although negativity of Bence-Jones proteinuria and a normal serum FLC ratio are frequently associated with the absence of MGRS, kidney biopsy, beyond its diagnostic and prognostic interest, remains the most discriminating test.

scholarly journals Crystalglobulinemia in Multiple Myeloma: A Rare Case Report of Survival and Renal Recovery

2020 ◽  
Vol 7 ◽  
pp. 205435812092262
Author(s):  
Angela Chou ◽  
Christopher Long ◽  
Leon Vonthethoff ◽  
Shir-Jing Ho ◽  
Franziska Pettit ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Acute Kidney Injury ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Kidney Biopsy ◽  
Rare Complication ◽  
Kidney Injury ◽  
Endothelial Damage ◽  
Cast Nephropathy ◽  
Rare Case Report

Rationale: Crystalglobulinemia is a rare complication of monoclonal gammopathy wherein crystallized immunoglobulins deposit in various organs causing occlusive vasculopathy, endothelial damage, and thrombosis. It should be differentiated from light chain cast nephropathy without crystalline nephropathy through timely diagnosis with a kidney biopsy. Presenting concerns of the patient: We report a case of a 74-year-old female with polyarthralgia, chest pain, petechial rash, and acute kidney injury. Diagnoses: Kidney biopsy revealed eosinophilic casts in the tubular lumen and similar occlusive crystalline deposits within the glomerular vasculature and interlobular arteries. Bone marrow biopsy and serum electrophoresis confirmed immunoglobulin G (IgG) κ multiple myeloma. Interventions: Dialysis was initiated for severe oligoanuric acute kidney injury. The patient was treated with 5 sessions of plasmapheresis and 11 cycles of clone reduction chemotherapy with CyBorD (cyclophosphamide, bortezomib, and dexamethasone). Outcomes: This patient achieved excellent kidney recovery and is no longer dialysis dependent. Teaching points: Crystalglobulinemia should be suspected in patients with rapidly progressive acute kidney injury and monoclonal gammopathy. Timely investigation with kidney biopsy to differentiate this condition from light chain cast nephropathy and initiation of appropriate treatment can lead to remission of disease and excellent recovery of kidney function.

Renal disease

2011 ◽  
pp. 631-656
Keyword(s):  
Chronic Kidney Disease ◽  
Acute Kidney Injury ◽  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Renal Disease ◽  
Nutritional Assessment ◽  
Kidney Injury ◽  
Nutritional Requirements ◽  
P Nutrition ◽  
Disease Stages

Introduction 632 Nutritional assessment 634 Malnutrition in renal disease 636 Nutritional considerations in chronic kidney disease 638 Nutrition in acute kidney injury 641 Nutrition in chronic kidney disease stages 3 and 4 642 Nephrotic syndrome 644 Nutritional requirements in dialysis 646 Nutritional requirements in haemodialysis ...

scholarly journals Acute Kidney Injury Associated with Minimal Change Nephrotic Syndrome in an Elderly Patient Successfully Treated with both Fluid Management and Specific Therapy Based on Kidney Biopsy Findings

2020 ◽  
Vol 10 (1) ◽  
pp. 42-50 ◽  
Author(s):  
Yuko Oyama ◽  
Yoichi Iwafuchi ◽  
Tetsuo Morioka ◽  
Ichiei Narita
Keyword(s):  
Elderly Patient ◽  
Acute Kidney Injury ◽  
Nephrotic Syndrome ◽  
Kidney Biopsy ◽  
Fluid Management ◽  
Kidney Injury ◽  
Oral Prednisolone ◽  
Minimal Change Nephrotic Syndrome ◽  
Minimal Change ◽  
Fluid Removal

Oliguric acute kidney injury (AKI) with minimal change nephrotic syndrome (MCNS) has long been recognized. Several mechanisms such as hypovolemia due to hypoalbuminemia and the nephrosarca hypothesis have been proposed. However, the precise mechanism by which MCNS causes AKI has not been fully elucidated. Herein, we describe an elderly patient with AKI caused by MCNS who fully recovered after aggressive volume withdrawal by hemodialysis and administration of a glucocorticoid. A 75-year-old woman presented with diarrhea and oliguria, and laboratory examination revealed nephrotic syndrome (NS) and severe azotemia. Fluid administration had no effect on renal dysfunction, and hemodialysis was initiated. Her renal function improved upon aggressive fluid removal through hemodialysis. Renal pathological findings revealed minimal change disease with faint mesangial deposits of IgA. After administration of methylprednisolone pulse therapy followed by oral prednisolone, she achieved complete remission from NS. The clinical course of this case supports the nephrosarca hypothesis regarding the mechanism of AKI caused by MCNS. Furthermore, appropriate fluid management and kidney biopsy are also important in elderly patients with AKI caused by NS.

scholarly journals P0508NEPHROTIC SYNDROME DUE TO HEAVY AND LIGHT CHAIN (AHL) AMYLOIDOSIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Csilla Markóth ◽  
László Bidiga ◽  
Piroska Pettendi ◽  
Éva Rékasi ◽  
László Ujhelyi ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Nephrotic Syndrome ◽  
Plasma Cell ◽  
Heavy Chain ◽  
Light Chain ◽  
Kidney Biopsy ◽  
Kidney Diseases ◽  
Screening Tests ◽  
Renal Outcome ◽  
Plasma Cell Dyscrasia

Abstract Background and Aims Kidney diseases with heavy chain deposition are rare, including AHL amyloidosis also. The mutation/deletion of the constant domain (CH1/CH2) of the heavy chain causing high tissue affinity seems most likely in its pathogenesis. The very low serum level is responsible for the difficult diagnosis, which is often based on kidney biopsy or laser microdissection / mass spectrometry. Method Case study of a 76-year-old male patient, examined in January, 2019. Results Besides treatment for Ménière syndrome and benign prostatic hyperplasia there was no other important event in patient’s history. Significant proteinuria and microscopic haematuria were observed from May 2016, but eGFR was 70 ml/min/1,73 m2 at that time. By April, 2018 nephrotic range proteinuria (10 g/day) with full nephrotic syndrome developed. Screening tests for cancer were negative. Despite symptomatic treatment, half year later eGFR decreased to 27 ml/min/1,73 m2, therefore he was referred to nephrology. Serum protein electrophoresis verified IgG lambda (8,1 g/l) and free lambda (0,5 g/l) monoclonal light chains, and in addition the possibility of IgG heavy chain accumulation. Urine electrophoresis showed also IgG lambda (1720,1 mg/l), and free lambda light chain (552,1 mg/l) monoclonality. Serum free lambda and kappa light chain ratio was 0,06, complement serology was normal. Kidney biopsy was done, which showed IgG heavy and light chain restriction, Congo red stain positivity and apple green birefringence under the polarized microscope in the expanded mesangium, in the interstitium and along the tubular basement membrane and the blood vessels. The electron microscope detected fibrillary deposits (10 nm) in the same structures, therefore diagnosis of AHL amyloidosis was established. He had no extrarenal symptoms. Bone marrow aspiration flow cytometry verified 1,11% plasma cell accumulation, 93% of them had pathological immunphenotype. Bone marrow morphology assay showed 30-40% plasma cell infiltration, and chromosome assay detected monoallelic deletion of IgH and MAF and gains of 1q region, suggesting myeloma multiplex in the background of AHL amyloidosis. VCD (bortezomib-cyclophosphamide-dexamethason) treatment was started, so far he has received 8 cycles. He is asymptomatic, proteinuria decreased, kidney function stabilized, eGFR 23 ml/min/1,73 m2. Conclusion only about 20 cases of AHL amyloidosis have been reported in the literature so far. In the context of longstanding kidney failure with nephrotic syndrome, we should consider renal disease associated with plasma cell dyscrasia also. If case of an AHL amyloidosis caused by myeloma multiplex, effective anti-plasma cell therapy can improve the hematological and the renal outcome.

Monoclonal immunoglobulin–mediated kidney disease: What is beyond amyloidosis in real practice?

2019 ◽  
Vol 3 (3) ◽  
pp. 105-112 ◽  
Author(s):  
Elena V Zakharova ◽  
Tatyana A Makarova ◽  
Ekaterina S Stolyarevich ◽  
Olga A Vorobyeva
Keyword(s):  
Kidney Disease ◽  
B Cell ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Monoclonal Immunoglobulin ◽  
B Cell Malignancies ◽  
Cast Nephropathy ◽  
Monoclonal Immunoglobulin Deposition Disease ◽  
Proximal Tubulopathy ◽  
Light Chain Proximal Tubulopathy

Background:Monoclonal immunoglobulin–mediated kidney disease with various patterns of damage may occur in patients with B-cell malignancies and non-malignant monoclonal gammopathies, and the latter are actually merged under the umbrella of monoclonal gammopathy of renal significance. Amyloidosis is the most well-known monoclonal immunoglobulin–related kidney damage. We focused on the rarer conditions and aimed to evaluate the non-amyloid spectrum of monoclonal immunoglobulin–mediated patterns of renal damage in real clinical practice.Methods:A single-center non-interventional retrospective study included 45 patients with pathology-proven non-amyloid monoclonal immunoglobulin–mediated kidney disease, followed during 2002–2018. Disease duration, proteinuria, serum creatinine, need for dialysis at the time of kidney biopsy, clinical diagnosis, and kidney pathology findings were analyzed.Results:No significant differences in the median age, disease duration at the time of biopsy, or main clinical presentation of kidney disease were found between patients with monoclonal gammopathy of renal significance and patients with B-cell malignancies. Pathology patterns like proliferative glomerulonephritis with monoclonal immunoglobulin deposits, membranous nephropathy, C3 glomerulopathy, cryoglobulinemic glomerulonephritis, and combinations of light chain proximal tubulopathy with monoclonal immunoglobulin deposition disease, and of C3 glomerulopathy with light chain proximal tubulopathy were found in monoclonal gammopathy of renal significance setting only. In contrast, light chain proximal tubulopathy alone, anti-glomerular basement glomerulonephritis, and combinations of cast nephropathy with light chain proximal tubulopathy, and cast nephropathy with monoclonal immunoglobulin deposition disease were associated with multiple myeloma only. Monoclonal immunoglobulin deposition disease, intracapillary monoclonal immunoglobulin M deposits, and cast nephropathy alone were seen in both settings.Conclusion:The presence of monoclonal gammopathy in patients with proteinuria and/or impaired kidney function demands kidney biopsy. Neither duration of kidney disease nor its clinical presentation allows differentiating malignant and non-malignant causes of monoclonal immunoglobulin–mediated renal damage. Several pathology patterns, even cast nephropathy, can be found both in cases of monoclonal gammopathy of renal significance and in cases of B-cell malignancies. Dual patterns of damage, including combinations of organized and non-organized deposits, or organized deposits with monoclonal immunoglobulin–induced damage without monoclonal immunoglobulin deposition, constitute up to 9%, mostly in multiple myeloma cases.

scholarly journals Kidney disease associated with plasma cell dyscrasias

Blood ◽  
2010 ◽  
Vol 116 (9) ◽  
pp. 1397-1404 ◽  
Author(s):  
Eliot C. Heher ◽  
Nelson B. Goes ◽  
Thomas R. Spitzer ◽  
Noopur S. Raje ◽  
Benjamin D. Humphreys ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Plasma Cell ◽  
Molecular Mechanisms ◽  
Kidney Injury ◽  
Light Chains ◽  
Free Light Chains ◽  
Monoclonal Immunoglobulin ◽  
Plasma Cell Dyscrasias ◽  
Made In

Plasma cell dyscrasias are frequently encountered malignancies often associated with kidney disease through the production of monoclonal immunoglobulin (Ig). Paraproteins can cause a remarkably diverse set of pathologic patterns in the kidney and recent progress has been made in explaining the molecular mechanisms of paraprotein-mediated kidney injury. Other recent advances in the field include the introduction of an assay for free light chains and the use of novel antiplasma cell agents that can reverse renal failure in some cases. The role of stem cell transplantation, plasma exchange, and kidney transplantation in the management of patients with paraprotein-related kidney disease continues to evolve.

scholarly journals Acute Kidney Injury In Hospitalized Patients Who Underwent Percutaneous Kidney Biopsy

2019 ◽  
Author(s):  
Henrique Pinheiro Konigsfeld ◽  
Thais Oliveira Claizoni Dos Santos ◽  
Tatiana Garcia Viana ◽  
Suzy Cristine Pereira ◽  
Gianna Mastroianni Kirsztajn ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Kidney Disease ◽  
Kidney Biopsy ◽  
Density Lipoprotein ◽  
Kidney Injury ◽  
Tertiary Hospital ◽  
Epidemiological Studies ◽  
Hospitalized Patients ◽  
Baseline Serum ◽  
Primary Indication

Abstract Background Performing a kidney biopsy is necessary to accurately diagnose diseases such as glomerulonephritis and tubulointerstitial nephritis, among other such conditions. These conditions predispose patients to chronic kidney disease, as well as acute kidney injury (AKI). Notably, most epidemiological studies describing AKI have not investigated this patient population. Methods Included patients admitted to the nephrology ward of a tertiary hospital who underwent percutaneous kidney biopsy. AKI was diagnosed based on the Kidney Disease: Improving Global Outcomes criteria. Results Of the 223 patients investigated, 140 (62.8%) showed AKI. Of these, 91 (65%), 19 (13.6%), and 30 (21.4%) presented with AKI classified as stages 1, 2, and 3, respectively. The primary indication for performing biopsy was nephrotic syndrome or nephrotic proteinuria (73 [52.1%] in the AKI vs. 51 [61.4%] in the non-AKI group, p=0.048). Focal segmental glomerulosclerosis was the most prevalent primary disease (24 [17.1%] in the AKI vs. 15 [18.0%] in the non-AKI group, p=0.150). Multivariate analysis of risk factors associated with AKI showed hemoglobin levels (odds ratio [OR] 0.805, 95% confidence interval [CI] 0.681–0.951, p=0.011), serum high-density lipoprotein cholesterol levels (HDL-c, OR 0.970, 95% CI 0.949–0.992, p=0.008), and baseline serum creatinine levels (OR 2.703, 95% CI 1.471–4.968, p=0.001) were significantly associated with AKI. Conclusions We observed a high incidence of AKI in hospitalized patients who underwent kidney biopsy to investigate their renal disease, particularly glomerulonephritis. Higher levels of hemoglobin and serum HDL-c were associated with a lower risk of AKI.

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

2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Gaetano Alfano ◽  
Alice Delrio ◽  
Francesco Fontana ◽  
Giacomo Mori ◽  
Silvia Cazzato ◽  
...  
Keyword(s):  
Kidney Disease ◽  
End Stage Renal Disease ◽  
Monoclonal Gammopathy ◽  
Kidney Biopsy ◽  
Clinical Manifestations ◽  
Kidney Injury ◽  
M Protein ◽  
Monoclonal Gammopathies ◽  
Stage Renal Disease ◽  
End Stage

Monoclonal gammopathies are associated with acute and chronic kidney injury. Nephrotoxicity of the secreted monoclonal (M)-protein is related to its biological properties and blood concentration. Little is known about epidemiology, clinical manifestations, and outcome of monoclonal gammopathies in patients with kidney disease. We retrospectively collected data about demographics, clinical manifestations, and renal histological lesions of all patients (n = 1334) who underwent kidney biopsy between January 2000 and March 2017. Monoclonal gammopathy was detected in 174 (13%) patients with a mean age of 66.4 ± 13.1 years. The spectrum of monoclonal gammopathies comprised monoclonal gammopathy of undetermined significate (MGUS) (52.8%), multiple myeloma (MM) (25.2%), primary amyloidosis (AL) (9.1%), smoldering MM (SMM) (4%), non-Hodgkin lymphoma (NHL) (6.8%), and Hodgkin lymphoma (HL) (1.7%). Monoclonal gammopathy of renal significance (MGRS) accounted for 6.5% in patients with MGUS and 14.2% in patients with SMM. Evaluation of kidney biopsy revealed that M-protein was directly involved in causing kidney injury in MM (93.1%). MM was the only gammopathy significantly associated with an increased risk of kidney injury (odds ratio [OR] = 47.5, CI 95%, 13.7–164.9; P ≤ 0.001 ). While there were no significant differences in the progression toward end-stage renal disease or dialysis P = 0.776 , monoclonal gammopathies were associated with a different risk of death P = 0.047 at the end of the follow-up. In conclusion, monoclonal gammopathy was a frequent finding (13%) in patients who underwent kidney biopsy. M-protein was secreted by both premalignant (56.8%) and malignant (43.2%) lymphoproliferative clones. Kidney biopsy had a key role in identifying MGRS in patients with MGUS (6.5%) and SMM (14.2%). Among monoclonal gammopathies, only MM was significantly associated with biopsy-proven kidney injury. The rate of end-stage renal disease or dialysis was similar among monoclonal gammopathies, whereas NHL, MM, and SMM showed a higher rate of deaths.

MO182CRYSTALLINE LIGHT CHAIN CAST NEPHROPATHY IS ASSOCIATED WITH EARLY MORTALITY IN PATIENTS WITH MULTIPLE MYELOMA

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Zishan Lin ◽  
Xu Zhang ◽  
Xiaojuan Yu ◽  
Suxia Wang ◽  
Xinan Cen ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Acute Kidney Injury ◽  
Median Time ◽  
Light Chain ◽  
Kidney Injury ◽  
Early Mortality ◽  
Monoclonal Immunoglobulin ◽  
Cast Nephropathy ◽  
Significant Difference ◽  
Ordinary Light

Abstract Background and Aims Light chain cast nephropathy is the most common paraprotein-associated kidney lesion in patients with multiple myeloma (MM). Rarely, light chain cast nephropathy could show crystalline appearance in patients with multiple myeloma, also known as crystalline light chain cast nephropathy. We here report the first retrospective study of crystalline light chain cast from a single centre. Method All native kidney biopsies were retrospectively studied in the Peking University First Hospital from 2000 to 2020. Newly diagnosed MM patients with were enrolled. Patients with light chain cast nephropathy at least one cast with crystalline appearance were identified as crystalline light chain cast nephropathy (Figure 1, n = 8), others were identified as ordinary light chain cast nephropathy (n = 18). Results The cohort of crystalline light chain cast nephropathy consisted of 6 men and 2 women with a median age of 59.5 (range, 41-73) years. All patients suffered from advanced multiple myeloma (1 with ISS staging II, 7 with ISS staging III) and acute kidney injury with a median eGFR of 5.59 (range, 2.27-26.04) mL/min/1.73m2. All patients except 1 required emergency dialysis at admission. Microhematuria was presented in 3 patients. Median proteinuria was 2.13 (rang, 0.83-3.59) g/24h and median serum albumin was 38.2 (30.7-46.7) g/L. No one presented with nephrotic syndrome. Monoclonal immunoglobulin, detected in all patients on serum protein immunofixation electrophoresis, was λ alone in 5 patients, κ alone in 1 patient, IgG λ in 1 patient, IgA λ in 1 patient. The 8 patients were followed up with a median time of 8 (range, 2-24) months. Three patients received VAD chemotherapy and 5 patients received bortezomib based regimens. At the time of last follow-up, 2 of 7 patients who needed emergency dialysis got rid of dialysis and the rest remained dialysis-dependent. Five patients died with a median time of 5 (range, 2-19) months, 2 patients achieved partial remission and 1 patient achieved complete remission. There was no significant difference in clinical features, treatments and main outcomes between crystalline light chain cast nephropathy patients and ordinary light chain cast nephropathy patients (Table 1). However, crystalline light chain cast nephropathy patients had higher early mortality than ordinary light chain cast nephropathy patients (50.0% vs 11.1%, p = 0.03). Conclusion Crystalline light chain cast nephropathy patients usually presented with acute kidney injury requiring emergency dialysis. Although various types of monoclonal immunoglobulin were detected, there was a dominance of the λ isotype. Compared to ordinary light chain cast nephropathy patients, crystalline light chain cast nephropathy patients had higher early mortality.

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