High cutoff versus high-flux haemodialysis for myelomacastnephropathy in patients receiving bortezomib-based chemotherapy (EuLITE): a phase 2 randomised controlled trial. In multiple myeloma, severe acute kidney injury due to myelomacastnephropathy is caused by pathogenic free light chain immunoglobulin in serum. High cutoff haemodialysis (HCO-HD) can remove large quantities of free light chain immunoglobulin from serum, but its effect on clinical outcomes is uncertain. We therefore aimed to assess whether HCO-HD could increase the frequency of renal recovery in patients with de novo multiple myeloma, severe acute kidney injury, and myelomacastnephropathy relative to treatment with standard high-flux haemodialysis (HF-HD). In this open-label, phase 2, multicentre, randomised
Effect of High-Cutoff Hemodialysis vs Conventional Hemodialysis on Hemodialysis Independence Among Patients With MyelomaCastNephropathy: A Randomized Clinical Trial. Cast nephropathy is the main cause of acute kidney injury in multiple myeloma and persistent reduction in kidney function strongly affects prognosis. Strategies to rapidly remove nephrotoxic serum-free light chains combined with novel antimyeloma agents have not been evaluated prospectively. To compare the hemodialysis independence rate among patients newly diagnosed with myelomacastnephropathy treated with hemodialysis using a high-cutoff dialyzer (with very large membrane pores and high permeability to immunoglobulin light chains) or a conventional high-flux dialyzer (with small pores and lower permeability). Randomized
Effectiveness of IHD with Adsorptive PMMA Membrane in MyelomaCastNephropathy: A Cohort Study. In patients with cast nephropathy and acute kidney injury (AKI) requiring dialysis, the reduction of serum free light chains (FLC) using chemotherapy and intensive hemodialysis (IHD) with a high cut-off filter may improve renal and patient outcomes. We evaluated the effectiveness of a combination castnephropathy, early FLC removal by IHD-PMMA combined with chemotherapy was associated with high rates of renal recovery and survival.
Paraprotein-Related Kidney Disease: Evaluation and Treatment of MyelomaCastNephropathy. Nearly 50% of patients with multiple myeloma develop renal disease, most commonly from AKI caused by cast nephropathy. Development of AKI is associated with poor 1-year survival and reduces the therapeutic options available to patients. There is a great need for more effective therapies. Cast nephropathy
Successful treatment of myelomacastnephropathy using bortezomib-based chemotherapy plus selective plasma exchange Myelomacastnephropathy is a major complication of multiple myeloma. Recent evidence has demonstrated that the earlier induction of bortezomib-based chemotherapy with plasma exchange (PE) provides better results for kidney function and patient survival. Due to its non-selectivity , PE with albumin replacement carries the risk of fibrinogen loss, leading to bleeding. We herein report a case of successful treatment of myelomacastnephropathy using bortezomib-based chemotherapy and selective PE. A 61-year-old woman who had a 20-year history of type II diabetes mellitus was admitted to our hospital for the evaluation of hypercalcemia, severe kidney dysfunction, and anemia
November Nephrology Web Episode - MyelomaCastNephropathy Renal Fellow Network: November Nephrology Web Episode - Myeloma Cast Nephropathyskip to main | skip to sidebarHome | About | Grants | Courses | RFN in the News Thursday, October 26, 2017November Nephrology Web Episode - Myeloma Cast NephropathyThe November 2017 nephrology web episode from Washington University is available for viewing a bit early to avoid conflicts with the upcoming #KidneyWk! This webisode is a short and basic lecture looking at myelomacastnephropathy. We start with a case, discuss the classic pathology, and go over the controversial role of plasma exchange for light chain removal.Posted byUnknownat1:12 PMEmail ThisBlogThis!Share to TwitterShare to FacebookShare to PinterestLabels:Tim YauNo comments:Post
Diagnostic accuracy of monoclonal antibody based serum immunoglobulin free light chain immunoassays in myelomacastnephropathy. undefined The development of serum immunoassays for the measurement of immunoglobulin free light chains has led to a paradigm shift in the diagnosis, assessment and monitoring of patients with plasma cell dyscrasias. The impact of these immunoassays which employ
Optimizing treatment strategies in myelomacastnephropathy: rationale for a randomized prospective trial. Renal failure is a frequent complication of multiple myeloma (MM) that strongly affects patient survival. Although a variety of renal diseases may be observed in MM, myelomacastnephropathy (MCN), a tubulo-interstitial disorder related to precipitation of a monoclonal light chain (LC
, hematuria, proteinuria, and hypocomplementemia. Kidney biopsy revealed ITG concomitant with κ myelomacastnephropathy. The glomerular microtubular deposits stained for κ and C3 only. Proteomic analysis of glomeruli and atypical casts detected Ig κ constant domain and a single VL variability subgroup (IGKV3) in both glomeruli and casts (without Ig γ, α, μ, or λ). C3, C5, C6, C7, and C9 were detected
Randomized Trial Comparing Double Versus Triple Bortezomib-Based Regimen in Patients With Multiple Myeloma and Acute Kidney Injury Due to Cast Nephropathy We report a multicenter controlled trial comparing renal recovery and tolerance profile of doublet versus triplet bortezomib-based regimens in patients with initial myelomacastnephropathy (CN) and acute kidney injury (AKI) without need
is exceeded.Glomerulopathic light-chains (G-LC) interact with mesangial cells and alter the mesangial homeostasis in 2 different ways, depending on whether G-LC is from a patient with LCCDD or amyloidosis. In contrast, the tubulopathic light chains (T-LC) from patients with myelomacastnephropathy do not significantly interact with mesangial cells and do not alter mesangial homeostasis. Some of these light chains the distal tubules and collecting ducts.Factors that might contribute to myelomacastnephropathy include the following: 1. 2. The direct toxicity of the intact light chains to tubular cells (compared with light chain fragment deposition in light chain deposition disease or amyloidosis) 3. 4. Protein complex formation in the distal nephron 5. 6. Tubular fluid pH 7. 8. A reduction in renal
Acute Renal Colic Due to Immunoglobulin Free Light Chain Kidney Stones: A Case Report of an Unusual Complication of Multiple Myeloma. Kidney failure is common in patients with a monoclonal gammopathy, most frequently due to hypercalcemia or myelomacastnephropathy. Immunoglobulin crystallization is an uncommon phenomenon that also results in kidney injury. We report the case of a 74-year-old man
survival rate of 60% . About 31% of newly diagnosed MM patients presented with renal impairment and about 6% to 13% need dialysis , which significantly increases morbidity and mortality, and limits treatment strategies . Most common renal disease in MM is myelomacastnephropathy that derived from secreted free light chains that combined with uromodulin to form cast which obstructs the distal tubules and presented with severe acute kidney injury . Renal biopsy is the only method to accurately diagnose myelomacastnephropathy. Uromodulin, also known as Tamm-Horsfall protein, is a kidney specific glycoprotein which is produced only by the epithelial cells lining the thick ascending limb (85%-90%) and early distal convoluted tubule (10%-15%), and is released to urine and blood . About 200 mg/day
: sepsis, heart diseases, polytrauma, liver disease, and cardiovascular surgery. The ICD-10 code corresponding to AKI (N17) was recorded in 30% of the cases with HA-AKI identified, and in this situation, 20% of the diagnoses associated with HA-AKI corresponded to kidney diseases such as tubulointerstitial nephritis, necrotizing vasculitis, or myelomacastnephropathy. Codes associated with HA-AKI
Combined proximal tubulopathy, crystal-storing histiocytosis, and cast nephropathy in a patient with light chain multiple myeloma. The diagnosis of myeloma, a plasma dyscrasia, often results from the workup of unexplained renal disease. Persistent renal failure in myeloma is commonly caused by tubular nephropathy due to circulating immunoglobulins and free light chains. Myelomacastnephropathy , and myelomacastnephropathy in a patient with κ light chain multiple myeloma. A 48-years-old man presented with uremia and anemia. Laboratory examination revealed low levels of serum IgG, IgA, and IgM. Serum and urine immunofixation electrophoresis showed a free κ monoclonal band. Bone marrow aspiration and biopsy revealed hypercellularity with marked plasmacytosis. Light microscopy revealed eosinophilic
of this retrospective multi-centre study was to compare the efficacy (renal recovery rate) of IHD and of standard haemodialysis (SHD) in patients with MM and dialysis-dependent acute kidney injury (AKI), concomitantly treated with bortezomib-based chemotherapy. We selected 41 patients with MM and dialysis-dependent AKI, most likely due to myelomacastnephropathy (MCN), and who were treated in eight French hospitals
Cisplatin-Induced Nephrotoxicity and HIV Associated Nephropathy: Mimickers of Myeloma-Like Cast Nephropathy Myelomacastnephropathy is an obstructing disorder of renal tubules, caused by precipitation of Bence Jones proteins. Myeloma-like cast nephropathy (MLCN) has been reported in the literature to occur in various primary renal and nonrenal diseases. We present a series of three rare cases with scattered high electron-dense globules. Myeloma-like cast nephropathy and true myelomacastnephropathy pose similar destructive effects on renal parenchyma. This new pattern of HIV-related nephropathy should be considered in HIV patients with MLCN, once monoclonal gammopathy is ruled out.
Trends in Survival and Renal Recovery in Patients with Multiple Myeloma or Light-Chain Amyloidosis on Chronic Dialysis. Monoclonal gammopathies (MGs) with renal involvement can lead to ESRD caused by myelomacastnephropathy (MCN), immunoglobulin light chain amyloidosis (ALA), or light-chain deposition disease (LCDD). Few studies have focused on the prognosis of patients with MG on chronic
is very poor. However, in this current period, survival is improving and the focus is on optimum use of novel chemotherapies and the evaluation of extra-corporeal therapies for removal of serum immunoglobulin light chains. RI in patients with MM is commonly associated with excess monoclonal free light chain (FLC) production; myelomacastnephropathy is the predominant renal pathology in patients countries. (1) A serum creatinine level >2 mg/dl has been reported in 16, 21, 24, and 33% of patients with MM in cohort studies from Japan, Europe, China, and Korea, respectively. A creatinine clearance rate <30 ml/min was observed in 30 and 15% of patients in Chinese and Western MM cohorts, respectively. The commonest cause of severe RI in patients with MM is myelomacastnephropathy. (2) The efficacy