Combined proximal tubulopathy, crystal-storing histiocytosis, and cast nephropathy in a patient with light chain multiple myeloma.

Abstract:

BACKGROUND: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. Myeloma cast nephropathy is characterized by crystalline precipitates of monoclonal light chains within distal tubules. Immunoglobulin crystallization rarely occurs intracellularly, within proximal tubular cells (light chain proximal tubulopathy) and interstitial histiocytes (crystal-storing histiocytosis). We present a case report of a rare simultaneous occurrence of light chain proximal tubulopathy, crystal-storing histiocytosis, and myeloma cast nephropathy in a patient with κ light chain multiple myeloma. CASE PRESENTATION: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 cuboid- and rhomboid-shaped crystals in the cytoplasm of proximal tubular epithelial cells, diffuse large mononuclear and multinuclear cells in the interstitium, and obstructed distal tubules with cast and giant cell reaction. Immunohistochemical examination indicated intense staining for κ light chains within casts, histiocytes, and tubular epithelial cells. Electron microscopy revealed electro-dense cuboid-, rhomboid-, or needle-shaped crystalline inclusions in proximal tubular epithelial cells and interstitial histiocytes. According to these results, we confirmed that this patient with myeloma exhibited simultaneous light chain proximal tubulopathy, crystal-storing histiocytosis, and myeloma cast nephropathy, which were attributed to monoclonal κ light chains. In addition to dialysis, the patient received induction chemotherapy with a combination of bortezomib, cyclophosphamide, and dexamethasone, followed by maintenance therapy with thalidomide. However, the patient did not regain renal function even when less than 5% plasma cells were detected in the bone marrow. CONCLUSION:To the best of our knowledge, this is the first report of simultaneous light chain proximal tubulopathy, crystal-storing histiocytosis, and myeloma cast nephropathy in κ light chain multiple myeloma.

journal_name

BMC Nephrol

journal_title

BMC nephrology

authors

Wu CK,Yang AH,Lai HC,Lin BS

doi

10.1186/s12882-017-0584-8

subject

Has Abstract

pub_date

2017-05-25 00:00:00

pages

170

issue

1

issn

1471-2369

pii

10.1186/s12882-017-0584-8

journal_volume

18

pub_type

杂志文章
  • Effect of tolvaptan on renal water and sodium excretion and blood pressure during nitric oxide inhibition: a dose-response study in healthy subjects.

    abstract:BACKGROUND:Tolvaptan is a selective vasopressin receptor antagonist. Nitric Oxide (NO) promotes renal water and sodium excretion, but the effect is unknown in the nephron's principal cells. In a dose-response study, we measured the effect of tolvaptan on renal handling of water and sodium and systemic hemodynamics, dur...

    journal_title:BMC nephrology

    pub_type: 杂志文章,随机对照试验

    doi:10.1186/s12882-017-0501-1

    authors: Al Therwani S,Rosenbæk JB,Mose FH,Bech JN,Pedersen EB

    更新日期:2017-03-13 00:00:00

  • Reference values for glomerular filtration rate in healthy Brazilian adults.

    abstract:BACKGROUND:Glomerular filtration rate (GFR) is the best index of renal function, but age, gender and ethnicity can putatively affect its values. The aim of this study was to establish reference values for GFR in healthy Brazilian subjects while taking these factors into account. METHODS:In this cross-sectional study, ...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/1471-2369-14-54

    authors: Soares AA,Prates AB,Weinert LS,Veronese FV,de Azevedo MJ,Silveiro SP

    更新日期:2013-02-28 00:00:00

  • Off-clamp partial nephrectomy has a positive impact on short- and long-term renal function: a systematic review and meta-analysis.

    abstract:BACKGROUND:Ongoing efforts are focused on shortening ischemia intervals as much as possible during partial nephrectomy to preserve renal function. Off-clamp partial nephrectomy (off-PN) has been a common strategy for to avoid ischemia in small renal tumors. Although studies comparing the advantages between off-PN with ...

    journal_title:BMC nephrology

    pub_type: 杂志文章,meta分析

    doi:10.1186/s12882-018-0993-3

    authors: Deng W,Liu X,Hu J,Chen L,Fu B

    更新日期:2018-07-31 00:00:00

  • The relation between dialysis-requiring acute kidney injury and recovery from end-stage renal disease: a national study.

    abstract:BACKGROUND:Approximately 4-6% of incident end stage renal disease (ESRD) patients in the U.S. recover enough kidney function to discontinue dialysis but there is considerable geographic variation. We undertook this study to investigate whether state-level variations in renal recovery among incident ESRD patients correl...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-019-1483-y

    authors: Chen Z,Lee BJ,McCulloch CE,Burrows NR,Heung M,Hsu RK,Pavkov ME,Powe NR,Saran R,Shahinian V,Hsu CY,Centers for Disease Control and Prevention Chronic Kidney Disease Surveillance Team.

    更新日期:2019-09-02 00:00:00

  • Designing a web-application to support home-based care of childhood CKD stages 3-5: qualitative study of family and professional preferences.

    abstract:BACKGROUND:There is a lack of online, evidence-based information and resources to support home-based care of childhood CKD stages 3-5. METHODS:Qualitative interviews were undertaken with parents, patients and professionals to explore their views on content of the proposed online parent information and support (OPIS) w...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/1471-2369-15-34

    authors: Swallow VM,Hall AG,Carolan I,Santacroce S,Webb NJ,Smith T,Hanif N

    更新日期:2014-02-18 00:00:00

  • Nephrolithiasis and risk of hypertension: a meta-analysis of observational studies.

    abstract:BACKGROUND:Observational studies have demonstrated an association between nephrolithiasis and hypertension. The aim of this meta-analysis was to summarize all available evidence. METHODS:PubMed, EMBASE, the Cochrane Central Register of Controlled Trials databases, and the reference lists of relevant articles were sear...

    journal_title:BMC nephrology

    pub_type: 杂志文章,meta分析

    doi:10.1186/s12882-017-0762-8

    authors: Shang W,Li Y,Ren Y,Yang Y,Li H,Dong J

    更新日期:2017-11-29 00:00:00

  • Unexpected recovery from longterm renal failure in severe diffuse proliferative lupus nephritis.

    abstract:BACKGROUND:Severe renal manifestation of systemic lupus erythematosus (SLE) is not uncommon and is associated with an indeterminate prognosis. Complete remission can be obtained, however, at least in the young when chronic lesions are absent and adequate anti-inflammatory therapy is immediately initiated. CASE PRESENT...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/1471-2369-13-81

    authors: Ross S,Benz K,Sauerstein K,Amann K,Dötsch J,Dittrich K

    更新日期:2012-08-06 00:00:00

  • Association of reduced inner retinal thicknesses with chronic kidney disease.

    abstract:BACKGROUND:Tissue derived biomarkers may offer utility as indicators of accumulated damage. Reduced thickness of retinal neuronal tissue and the vascular choroid have previously been associated with vascular damage and diabetes. We evaluated associations between retinal thickness, retinal microvascular and choroidal me...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-019-1679-1

    authors: Paterson EN,Ravindran ML,Griffiths K,Le Velly CA,Cardwell CC,McCarter RV,Nicol P,Chhablani JK,Rasheed MA,Vupparaboina KK,MacGillivray TJ,Harbinson M,Maxwell AP,Hogg RE,McKay GJ

    更新日期:2020-01-31 00:00:00

  • Urinary albumin excretion in healthy adults: a cross sectional study of 24-hour versus timed overnight samples and impact of GFR and other personal characteristics.

    abstract:BACKGROUND:Urinary albumin can be measured in 24 h or spot samples. The 24 h urinary albumin excretion rate is considered the gold standard, but is cumbersome to collect. Instead, often an overnight sample is collected, and adjusted for dilution. Proxies for 24 h excretion rate have been studied in diabetics, but seldo...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/1471-2369-16-8

    authors: Fagerstrom P,Sallsten G,Akerstrom M,Haraldsson B,Barregard L

    更新日期:2015-01-24 00:00:00

  • Estimation of renal function by CKD-EPI versus MDRD in a cohort of HIV-infected patients: a cross-sectional analysis.

    abstract:BACKGROUND:Accurately determining renal function is essential for clinical management of HIV patients. Classically, it has been evaluated by estimating glomerular filtration rate (eGFR) with the MDRD-equation, but today there is evidence that the new Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation ...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-017-0470-4

    authors: Cristelli MP,Cofán F,Rico N,Trullàs JC,Manzardo C,Agüero F,Bedini JL,Moreno A,Oppenheimer F,Miro JM,CKD-H. Clinic Investigators.

    更新日期:2017-02-10 00:00:00

  • Safety and adequacy of percutaneous kidney biopsy performed by nephrology trainees.

    abstract:BACKGROUND:Recently there has been a progressive loss of specialty related skills for nephrologists. Among the skills we find the kidney biopsy that has a central role in diagnosis of renal parenchymal disease. One of the causes might be the belief that the kidney biopsy should be performed only in larger Centers which...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-017-0796-y

    authors: Esposito V,Mazzon G,Baiardi P,Torreggiani M,Semeraro L,Catucci D,Colucci M,Mariotto A,Grosjean F,Bovio G,Esposito C

    更新日期:2018-01-15 00:00:00

  • Relationship between early proteinuria and long term outcome of kidney transplanted patients from different decades of donor age.

    abstract:BACKGROUND:Proteinuria after kidney transplantation portends a worse graft survival. However the magnitude of proteinuria related to patient and graft survival and its correlation with donor and recipient characteristics are poorly explored. METHODS:This study investigated the impact of post transplant proteinuria in ...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-019-1635-0

    authors: Diena D,Messina M,De Biase C,Fop F,Scardino E,Rossetti MM,Barreca A,Verri A,Biancone L

    更新日期:2019-12-02 00:00:00

  • Effect of calcitriol on serum hepcidin in individuals with chronic kidney disease: a randomized controlled trial.

    abstract:BACKGROUND:Anemia is highly prevalent in chronic kidney disease (CKD). Elevated hepcidin concentrations are an important mediator of disordered iron metabolism, a key mechanism underlying anemia of CKD. Vitamin D was recently shown to reduce serum hepcidin concentrations in healthy individuals. We examined whether trea...

    journal_title:BMC nephrology

    pub_type: 杂志文章,随机对照试验

    doi:10.1186/s12882-018-0823-7

    authors: Panwar B,McCann D,Olbina G,Westerman M,Gutiérrez OM

    更新日期:2018-02-09 00:00:00

  • The relative importance of frailty, physical and cardiovascular function as exercise-modifiable predictors of falls in haemodialysis patients: a prospective cohort study.

    abstract:BACKGROUND:Stage 5 chronic kidney disease (CKD-5) patients on haemodialysis (HD) are at high risk of accidental falls. Previous research has shown that frailty is one of the primary contributors to the increased risk of falling in this clinical population. However, HD patients often present with abnormalities of cardio...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-020-01759-z

    authors: Zanotto T,Mercer TH,van der Linden ML,Rush R,Traynor JP,Petrie CJ,Doyle A,Chalmers K,Allan N,Shilliday I,Koufaki P

    更新日期:2020-03-14 00:00:00

  • RhoA deficiency disrupts podocyte cytoskeleton and induces podocyte apoptosis by inhibiting YAP/dendrin signal.

    abstract:BACKGROUND:Podocyte apoptosis is a major mechanism that leads to proteinuria in many kidney diseases. However, the concert mechanisms that cause podocyte apoptosis in these kidney diseases are not fully understood. RhoA is one of Rho GTPases that has been well studied and plays a key role in regulating cytoskeletal arc...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-016-0287-6

    authors: Huang Z,Zhang L,Chen Y,Zhang H,Yu C,Zhou F,Zhang Z,Jiang L,Li R,Ma J,Li Z,Lai Y,Lin T,Zhao X,Zhang Q,Zhang B,Ye Z,Liu S,Wang W,Liang X,Liao R,Shi W

    更新日期:2016-07-07 00:00:00

  • Arg913Gln variation of SLC12A3 gene is associated with diabetic nephropathy in type 2 diabetes and Gitelman syndrome: a systematic review.

    abstract:BACKGROUND:Diabetic nephropathy is a global common cause of chronic kidney disease and end-stage renal disease. A lot of research has been conducted in biomedical sciences, which has enhanced understanding of the pathophysiology of diabetic nephropathy and has expanded the potential available therapies. An increasing n...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-019-1590-9

    authors: De la Cruz-Cano E,Jiménez-González CDC,Morales-García V,Pineda-Pérez C,Tejas-Juárez JG,Rendón-Gandarilla FJ,Jiménez-Morales S,Díaz-Gandarilla JA

    更新日期:2019-10-28 00:00:00

  • Trends in anemia care in non-dialysis-dependent chronic kidney disease (CKD) patients in the United States (2006-2015).

    abstract:BACKGROUND:The objective of the study was to examine overall anemia management trends in non-dialysis patients with chronic kidney disease (CKD) from 2006 to 2015, and to evaluate the impact of Trial to Reduced Cardiovascular Events with Ananesp Therapy (TREAT)'s study results (October 2009) and the US Food and Drug Ad...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-018-1119-7

    authors: Park H,Liu X,Henry L,Harman J,Ross EA

    更新日期:2018-11-09 00:00:00

  • Excessive diagnostic testing in acute kidney injury.

    abstract:BACKGROUND:The patterns, performance characteristics, and yield of diagnostic tests ordered for the evaluation of acute kidney injury (AKI) have not been rigorously evaluated. METHODS:We characterized the frequency of AKI diagnostic testing for urine, blood, radiology, and pathology tests in all adult inpatients who w...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-016-0224-8

    authors: Leaf DE,Srivastava A,Zeng X,McMahon GM,Croy HE,Mendu ML,Kachalia A,Waikar SS

    更新日期:2016-01-15 00:00:00

  • Inadequate dietary energy intake associates with higher prevalence of metabolic syndrome in different groups of hemodialysis patients: a clinical observational study in multiple dialysis centers.

    abstract:BACKGROUND:Metabolic syndrome (MetS) has been established as a risk for cardiovascular diseases and mortality in hemodialysis patients. Energy intake (EI) is an important nutritional therapy for preventing MetS. We examined the association of self-reported dietary EI with metabolic abnormalities and MetS among hemodial...

    journal_title:BMC nephrology

    pub_type: 杂志文章,多中心研究

    doi:10.1186/s12882-018-1041-z

    authors: Duong TV,Wong TC,Chen HH,Chen TW,Chen TH,Hsu YH,Peng SJ,Kuo KL,Liu HC,Lin ET,Wang CS,Tseng IH,Feng YW,Chang TY,Su CT,Yang SH

    更新日期:2018-09-19 00:00:00

  • Soluble Klotho is associated with mortality and cardiovascular events in hemodialysis.

    abstract:BACKGROUND:Klotho is a transmembrane protein acting as a co-receptor for FGF-23 and thus exerts clinical actions on mineral metabolism. The association of secreted Klotho with outcomes in CKD patients is unclear. This study examined the relation between plasma Klotho and cardiovascular events in dialysis patients, acco...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-019-1391-1

    authors: Memmos E,Sarafidis P,Pateinakis P,Tsiantoulas A,Faitatzidou D,Giamalis P,Vasilikos V,Papagianni A

    更新日期:2019-06-11 00:00:00

  • Interventional study to improve adherence to phosphate binder treatment in dialysis patients.

    abstract:BACKGROUND:Adherence to phosphate binder treatment is important to prevent high serum phosphate level in chronic dialysis patients. We therefore wanted to investigate patient knowledge, beliefs about and adherence to phosphate binders among these patients and assess whether one-to-one pharmacist-led education and couns...

    journal_title:BMC nephrology

    pub_type: 临床试验,杂志文章

    doi:10.1186/s12882-019-1334-x

    authors: Hjemås BJ,Bøvre K,Mathiesen L,Lindstrøm JC,Bjerknes K

    更新日期:2019-05-17 00:00:00

  • Mortality in dialysis patients may not be associated with ESA dose: a 2-year prospective observational study.

    abstract:BACKGROUND:Anaemia of chronic kidney disease increases the risk of death and adverse events, but can be managed using erythropoiesis stimulating agents (ESAs). However, recent evidence suggests that targeting a higher haemoglobin concentration ([Hb]) increases mortality risk, and both higher [Hb] targets and ESA doses ...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/1471-2369-13-40

    authors: McMahon LP,Cai MX,Baweja S,Holt SG,Kent AB,Perkovic V,Leikis MJ,Becker GJ

    更新日期:2012-06-15 00:00:00

  • Identification of biomarkers and pathways in hypertensive nephropathy based on the ceRNA regulatory network.

    abstract:BACKGROUND:Hypertensive nephropathy (HTN) is a kind of renal injury caused by chronic hypertension, which seriously affect people's life. The purpose of this study was to identify the potential biomarkers of HTN and understand its possible mechanisms. METHODS:The dataset numbered GSE28260 related to hypertensive and n...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-020-02142-8

    authors: Wang Z,Liu Z,Yang Y,Kang L

    更新日期:2020-11-11 00:00:00

  • Renal neutrophil gelatinase associated lipocalin expression in lipopolysaccharide-induced acute kidney injury in the rat.

    abstract:BACKGROUND:Neutrophil gelatinase associated lipocalin (NGAL) is a highly predictive biomarker of acute kidney injury. To understand the role of NGAL in renal injury during sepsis, we investigated the temporal changes and biological sources of NGAL in a rat model of acute kidney injury, and explored the relationship bet...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/1471-2369-13-25

    authors: Han M,Li Y,Liu M,Li Y,Cong B

    更新日期:2012-06-27 00:00:00

  • High serum bicarbonate level within the normal range prevents the progression of chronic kidney disease in elderly chronic kidney disease patients.

    abstract:BACKGROUND:Metabolic acidosis leads to chronic kidney disease (CKD) progression. The guidelines recommend a lower limit of serum bicarbonate level, but no upper limit. For serum bicarbonate level to be clinically useful as a therapeutic target marker, it is necessary to investigate the target serum bicarbonate level wi...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/1471-2369-14-4

    authors: Kanda E,Ai M,Yoshida M,Kuriyama R,Shiigai T

    更新日期:2013-01-09 00:00:00

  • Should patients older than 65 years be offered a second kidney transplant?

    abstract:BACKGROUND:Age and number of recipients in need of kidney re-transplantation are increasing. Re-transplantation practices and outcomes in elderly recipients are not previously explored. We aimed to retrospectively evaluate the outcomes of recipients 65 years and older receiving their second deceased donor allograft. M...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-016-0426-0

    authors: Heldal K,Hartmann A,Lønning K,Leivestad T,Reisæter AV,Line PD,Holdaas H,Midtvedt K

    更新日期:2017-01-11 00:00:00

  • Time of injury affects urinary biomarker predictive values for acute kidney injury in critically ill, non-septic patients.

    abstract:BACKGROUND:The predictive value of acute kidney injury (AKI) urinary biomarkers may depend on the time interval following tubular injury, thereby explaining in part the heterogeneous performance of these markers that has been reported in the literature. We studied the influence of timing on the predictive values of tub...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/1471-2369-14-273

    authors: de Geus HR,Fortrie G,Betjes MG,van Schaik RH,Groeneveld AB

    更新日期:2013-12-09 00:00:00

  • Rationale and design of the Sodium Lowering In Dialysate (SoLID) trial: a randomised controlled trial of low versus standard dialysate sodium concentration during hemodialysis for regression of left ventricular mass.

    abstract:BACKGROUND:The current literature recognises that left ventricular hypertrophy makes a key contribution to the high rate of premature cardiovascular mortality in dialysis patients. Determining how we might intervene to ameliorate left ventricular hypertrophy in dialysis populations has become a research priority. Reduc...

    journal_title:BMC nephrology

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1186/1471-2369-14-149

    authors: Dunlop JL,Vandal AC,de Zoysa JR,Gabriel RS,Haloob IA,Hood CJ,Matheson PJ,McGregor DO,Rabindranath KS,Semple DJ,Marshall MR

    更新日期:2013-07-15 00:00:00

  • Chronic kidney disease of uncertain aetiology: adding vital piece of information to the national project team report of Sri Lanka.

    abstract::In a recent study published by the National Project Team on chronic kidney diseases of unknown origin in Sri Lanka, identified cadmium as a major risk factor but strong conclusions were not made as the identified environmental toxins were within the permissible levels.Sri Lankan food consumption pattern is different s...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-015-0211-5

    authors: Jayalal TB

    更新日期:2015-12-23 00:00:00

  • Care of the dialysis patient: Primary provider involvement and resource utilization patterns - a cohort study.

    abstract:BACKGROUND:Efficient and safe delivery of care to dialysis patients is essential. Concerns have been raised regarding the ability of accountable care organizations to adequately serve this high-risk population. Little is known about primary care involvement in the care of dialysis patients. This study sought to describ...

    journal_title:BMC nephrology

    pub_type: 杂志文章

    doi:10.1186/s12882-017-0728-x

    authors: Thorsteinsdottir B,Ramar P,Hickson LJ,Reinalda MS,Albright RC,Tilburt JC,Williams AW,Takahashi PY,Jeffery MM,Shah ND

    更新日期:2017-10-25 00:00:00