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cast nephropathy amyloidosis

Josephine M Vos, Joshua Gustine, Helmut G Rennke, Zachary Hunter, Robert J Manning, Toni E Dubeau, Kirsten Meid, Monique C Minnema, Marie-Jose Kersten, Steven P Treon, Jorge J Castillo
The incidence and prognostic impact of nephropathy related to Waldenström macroglobulinaemia (WM) is currently unknown. We performed a retrospective study to assess biopsy-confirmed WM-related nephropathy in a cohort of 1391 WM patients seen at a single academic institution. A total of 44 cases were identified, the estimated cumulative incidence was 5·1% at 15 years. There was a wide variation in kidney pathology, some directly related to the WM: amyloidosis (n = 11, 25%), monoclonal-IgM deposition disease/cryoglobulinaemia (n = 10, 23%), lymphoplasmacytic lymphoma infiltration (n = 8, 18%), light-chain deposition disease (n = 4, 9%) and light-chain cast nephropathy (n = 4, 9%), and some probably related to the WM: thrombotic microangiopathy (TMA) (n = 3, 7%), minimal change disease (n = 2, 5%), membranous nephropathy (n = 1, 2%) and crystal-storing tubulopathy (n = 1, 2%)...
July 29, 2016: British Journal of Haematology
Disha Arora, Mayank Uppal, Vindu Amitabh, Usha Agrawal
Light Chain Deposition Disease (LCDD) is a rare disease characterized by deposition of monoclonal non-amyloid light chains in multiple organs. We report an unusual histologic manifestation of LCDD in a 55-year-old female patient, who presented with nephrotic syndrome and an increased serum creatinine. This case of LCDD had features of cast nephropathy on biopsy which is diagnostic of myeloma kidney, when the patient was clinically asymptomatic. Serum electrophoresis showed no abnormal band. There was no other evidence of a B-cell clonal disorder or amyloidosis...
May 2016: Journal of Clinical and Diagnostic Research: JCDR
Imen Gorsane, S Barbouch, M Mayara, K Ben Abdelghani, R Goucha, F Ben Hamida, H Hedri, T Ben Abdallah
To determine the features of the different forms of kidney diseases associated with multiple myeloma (MM), we retrospectively studied 144 patients with kidney injury and MM at our institute from 1974 to 2014. The mean age of the patients was 60.1 years and the male:female ratio was 1.25. Renal disease was concomitant with the discovery of MM in 92% of cases. The mean follow-up of our patients was 2.1 years. Initial renal insufficiency was found in 131 (91%) patients. Cast nephropathy of the distal tubule was found in 110 (26%) patients, renal amyloidosis in 16 (11...
May 2016: Saudi Journal of Kidney Diseases and Transplantation
R Nada, A Kumar, V G Kumar, K L Gupta, K Joshi
Renal biopsy interpretation requires histopathology, direct immunofluorescence (DIF) and electron microscopy. Formalin-fixed, paraffin-embedded tissue (FFPE) sent for light microscopy can be used for DIF after antigen retrieval. However, complement staining has not been satisfactory. We standardized DIF using proteinase-K for antigen retrieval in FFPE renal biopsies. A pilot study was conducted on known cases of membranous glomerulonephritis (MGN), membranoproliferative type-1 (MPGN-1), immunoglobulin A nephropathy (IgAN), and anti-glomerular basement disease (anti-GBM)...
May 2016: Indian Journal of Nephrology
Yu-Tai Su, Xin-Sheng Xie, Hui Sun, Jie Ma, Ding-Ming Wan, Yan-Fang Liu
OBJECTIVE: To explore the clinical features of multiple myeloma with different renal pathology, and to evaluate its prognosis. METHODS: Clinical features and prognosis of 46 multiple myeloma patients with different renal pathology were analyzed retrospectively. According to renal pathology, the 46 patients were divided into 3 groups: cast nephropathy (24 cases), amyloidosis (15 cases) and other type (7 cases). RESULTS: By durie-Salmon staging system, 70...
April 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
Benoit Brilland, Johnny Sayegh, Anne Croue, Frank Bridoux, Jean-François Subra, Jean-François Augusto
Light chain deposition disease (LCDD) is a rare multisystemic disorder associated with plasma cell proliferation. It mainly affects the kidney, but liver and heart involvement may occur, sometimes mimicking the picture of systemic amyloidosis. Liver disease in LCDD is usually asymptomatic and exceptionally manifests with severe cholestatic hepatitis. We report the case of a 66-year-old female with κ-LCDD and cast nephropathy in the setting of symptomatic multiple myeloma who, after a first cycle of bortezomib-dexamethasone chemotherapy, developed severe and rapidly worsening intrahepatic cholestasis secondary to liver κ-light chain deposition...
March 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
Sanjeev Sethi, Fernando C Fervenza, S Vincent Rajkumar
PURPOSE OF REVIEW: Monoclonal gammopathies result from an overt malignant process, such as multiple myeloma, or a premalignant process, such as monoclonal gammopathy of undetermined significance. The kidney is often affected in the setting of a monoclonal gammopathy. The term 'monoclonal gammopathy of renal significance (MGRS)' was recently introduced to draw attention to renal diseases related to the monoclonal gammopathy. In this review, we define the pathology of these monoclonal gammopathy-associated kidney diseases...
March 2016: Current Opinion in Nephrology and Hypertension
Hideki Kato, Yoshihide Fujigaki, Shinichiro Asakawa, Yutaka Yamaguchi, Hiroshi Uozaki, Atsushi Komatsuda, Michio Nagata, Shunya Uchida
Multiple myeloma presents with various kidney injuries, including cast nephropathy, light chain deposition disease, and amyloidosis. Cast nephropathy is the most common form and mostly consists of monoclonal immunoglobulin light chains with Tamm-Horsfall protein. Immunoglobulin light chain (AL) amyloidosis may affect all compartments of the kidney, but it is rare in the tubuli. We herein present a rare case with rapid progression of renal failure caused by the co-occurrence of intratubular amyloidosis and cast nephropathy due to multiple myeloma...
2015: Internal Medicine
I G Rekhtina, E V Zakharova, E S Stoliarevich, M N Sinitsina, E N Denisova
Despite of the fact that their clinical manifestations are similar, AL-amyloidosis (AL-A) and light chain deposition disease (LCDD) are individual nosological entities in view of considerable differences in their pathogenesis and pathomorphology. The paper describes a rare case of the concurrence of LCDD and AL-A in a patient with multiple myeloma. Clinically, there was dialysis-dependent renal failure, flail leg syndrome, myocardiopathy, and rhabdomyolysis. At the disease onset, his nephrobiopsy specimen could diagnose LCDD and myeloma or cast nephropathy...
2015: Terapevticheskiĭ Arkhiv
Sophie Chauvet, Frank Bridoux, Laure Ecotière, Vincent Javaugue, Christophe Sirac, Bertrand Arnulf, Antoine Thierry, Nathalie Quellard, Serge Milin, Sébastien Bender, Jean-Michel Goujon, Arnaud Jaccard, Jean-Paul Fermand, Guy Touchard
BACKGROUND: Kidney diseases associated with immunoglobulin M (IgM) monoclonal gammopathy are poorly described, with few data for patient outcomes and renal response. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: 35 patients from 8 French departments of nephrology were retrospectively studied. Inclusion criteria were: (1) detectable serum monoclonal IgM, (2) estimated glomerular filtration rate (eGFR) < 60mL/min/1.73m(2) and/or proteinuria with protein excretion > 0...
November 2015: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Alexandra Presle, Jean-Philippe Bertocchio, Nathalie Schneider, François-Xavier Maquart, Laurent Ramont, Jean-Baptiste Oudart
Serum protein electrophoresis is commonly used in case of acute or chronic renal failure. It can lead to the etiologic diagnosis by detecting monoclonal gammopathies which are frequently complicated by renal failure, such as cast nephropathy, Randall's disease or amyloidosis, or to explore an associated inflammatory syndrome. We report the occurrence of two monoclonal components in a patient without any monoclonal component 10 days earlier. The sudden appearance of these two monoclonal components associated to the context of sepsis of urinary origin suggested the diagnosis of transient monoclonal gammopathy...
March 2015: Annales de Biologie Clinique
K K Gowda, K Joshi, R Nada, R Ramachandran, M Sachdeva
The renal diseases most frequently associated with myeloma include cast nephropathy (CN), amyloidosis and monoclonal immunoglobulin deposition disease. Light chain proximal tubulopathy (LCPT) is reported less frequently. Majority of the cases with κ-restriction present with Fanconi syndrome (FS) and show crystals in proximal tubular epithelial cytoplasm. In contrast, those with λ-restriction are infrequently associated with FS and show cytoplasmic vacuolations in proximal tubular epithelial cytoplasm. Combination of morphologies in kidney affected by plasma cell dyscrasias is rare and co-existence of LCPT and CN is one of the rarest...
March 2015: Indian Journal of Nephrology
Turki Al-Hussain, Maged H Hussein, Hadeel Al Mana, Mohammed Akhtar
Monoclonal gammopathy is produced by neoplastic or non-neoplastic expansion of a clone of plasma cells or B lymphocytes. Monoclonal gammopathy of unknown significance is characterized by low levels of the monoclonal protein and a relatively small population of clonal lymphocytes or plasma cells in the bone marrow. In these cases, the patient is asymptomatic with no evidence of overt myeloma or lymphoma. The abnormal serum protein may be present as a complete immunoglobulin molecule or may consist of ≥1 of its components such as light chains or heavy chains...
March 2015: Advances in Anatomic Pathology
Naoto Takahashi
Renal impairment (RI) is a common complication and it impacts on a survival of patients with systemic multiple myeloma (MM). In a cohort study in Akita, about 20-26% patients with MM showed RI at diagnosis and it was independently associated with overall survival. Cast nephropathy, amyloidosis, and monoclonal immunoglobulin deposition disease (Randall type) are known as three pathological findings of kidney in MM patients with RI. Recently, the term monoclonal gammopathy of renal significance (MGRS) was proposed because of a causal relationship between the monoclonal gammopathy and the renal damage...
January 2015: Nihon Rinsho. Japanese Journal of Clinical Medicine
Guillermo A Herrera
CONTEXT: Lesions associated with monoclonal light and heavy chains display a variety of glomerular, tubular interstitial, and vascular manifestations. While some of the entities are well recognized, including light and heavy chain deposition diseases, AL (light chain) and AH (heavy chain) amyloidosis, and light chain ("myeloma") cast nephropathy, other lesions centered on proximal tubules are much less accurately identified, properly diagnosed, and adequately understood in terms of pathogenesis and molecular mechanisms involved...
October 2014: Archives of Pathology & Laboratory Medicine
Tomasz Stompór, Agnieszka Perkowska-Ptasińska, Małgorzata Wojciechowska, Katarzyna Zając, Agnieszka Chmielewska-Badziąg, Anna Pawłowska
Multiple myeloma (MM) is the second most common hematological malignancy, with an annual incidence in Europe and the USA of about 4-6 cases per 100,000. Several forms of renal disease are found in the course of MM, including: cast nephropathy, light chain (LC) deposition disease and primary amyloidosis. Less frequent forms include: acute and chronic tubulopathies, neoplastic plasma cell infiltration and interstitial nephritis. In this paper, we discuss a case of 53-year-old male patient with MM who presented with massive proteinuria (24 g/24 h), mild renal insufficiency (eGFR 43 mL/min), and Fanconi-like syndrome (as reflected by normoglycemic glycosuria)...
June 2014: Renal Failure
Shreeram Akilesh, Astier Alem, Roberto F Nicosia
Lymphoplasmacytic neoplasms cause a wide range of injuries to the kidney exemplified by light chain cast nephropathy and amyloidosis. Filtered paraproteins can also accumulate within kidney cells and cause direct cytotoxic injury. Rarely, paraproteins that are resistant to proteolysis can crystallize within proximal tubules and cause acute tubular injury. In contrast, accumulation of crystallized paraproteins in other kidney cells, especially podocytes, is exceptional. Here, we report the finding of crystalline inclusions within podocytes and proximal tubules in a patient who presented with a combined nephrotic syndrome and Fanconi syndrome...
April 2014: Human Pathology
Diana Spinelli, Dario Consonni, Giuseppe Garigali, Giovanni B Fogazzi
BACKGROUND: Casts are well known components of the urinary sediment. For most casts, the clinical associations are known and demonstrated, while for waxy casts they are totally unknown. METHODS: Prospective study for the search and count of waxy casts in the urinary sediment of patients with different types of glomerular diseases. RESULTS: Waxy casts were found in 39 out of 287 patients (13.6%), mostly in low number (1 to 9 out of 100 casts evaluated/sample)...
September 23, 2013: Clinica Chimica Acta; International Journal of Clinical Chemistry
J Gerth, H Sigusch, N Illner, M Busch, L-O Muegge, T Lehmann, G Wolf
BACKGROUND: An impaired renal function in light chain associated disorders may be caused by myeloma cast nephropathy (MCN) but also by AL-amyloidosis (AL-A) and monoclonal immundeposition disease (MIDD). PATIENTS AND METHODS: In a monocentric, retrospective analysis, patients suffering from multiple myeloma (MM) (n = 392) requiring medical therapy, AL-A (n = 53) or MIDD (n = 12) diagnosed between 1996 and 2008 were evaluated for renal insufficiency. The different patient cohorts were compared in terms of their clinical course and outcome...
February 2013: Deutsche Medizinische Wochenschrift
Yun Zhong, Zhen-gang Yuan, Wei-jun Fu, Fan Zhou, Chun-yang Zhang, Wen-hao Zhang, Jian Hou
OBJECTIVE: To investigate the value of serum cystatin C (Cys-C), urinary Cys-C, urinary retinol binding protein (RBP) and urinary neutrophil gelatinase-associated lipocalin (NGAL) in the early assessment of multiple myeloma (MM) and their characteristic changes in different pathological types of renal impairment. METHODS: According to glomerular filtration rate (eGFR), the patients were divided into two groups, of which marked group A with normal renal function, the other marked group B with abnormal renal function...
October 2012: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
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