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Paraprotein

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https://www.readbyqxmd.com/read/28106236/chorioretinal-folds-in-a-patient-with-multiple-myeloma-treated-with-stem-cell-transplant
#1
Jia S Poon, Kaveh Vahdani, Adam P Booth
PURPOSE: To report an unusual case of bilateral choroidal folds related to multiple myeloma. METHODS: In this case report, fundus photography, fundus fluorescein angiography, fundus autofluorescence, and B-ultrasonography were used to assess posterior segment changes. RESULTS: A 55-year-old woman with history of multiple myeloma was found to have abnormal fundi. Clinical examination and investigations confirmed chorioretinal folds, which was considered to be related to myeloma...
January 16, 2017: European Journal of Ophthalmology
https://www.readbyqxmd.com/read/28101022/necrobiotic-xanthogranuloma-in-a-patient-with-multiple-myeloma
#2
Marlies Wruhs, Robert Feldmann, Isabelle Sawetz, Friedrich Breier, Andreas Steiner
An 82-year-old woman presented with a 9-month history of multiple, well-defined skin lesions on her neck and upper chest, progressively increasing in size. Histological examination of a skin biopsy showed a regular epidermis. In the dermis, granulomatous changes with central necrobiosis were found which extended focally into the subcutaneous fat. The necrobiotic areas were surrounded by Touton cells and foreign-body giant cells. Laboratory analysis revealed leucopenia. Serum electrophoresis and immunofixation disclosed the presence of an IgG-λ paraprotein...
September 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/28076910/treatment-of-patients-with-waldenstr%C3%A3-m-macroglobulinaemia-clinical-practice-guidelines-from-the-myeloma-foundation-of-australia-medical-and-scientific-advisory-group
#3
Dipti Talaulikar, Constantine S Tam, Douglas Joshua, Joy Phoebe Ho, Jeff Szer, Hang Quach, Andrew Spencer, Simon Harrison, Peter Mollee, Andrew W Roberts, Noemi Horvath, Cindy Lee, Andrew Zannettino, Ross Brown, Bradley Augustson, Wilfrid Jaksic, John Gibson, Anna Kalff, Anna Johnston, Judith Trotman, Akash Kalro, George Grigoriadis, Chris Ward, H Miles Prince
Waldenström macroglobulinaemia (WM) is an indolent B-cell malignancy characterised by the presence of immunoglobulin M (IgM) paraprotein and bone marrow infiltration by clonal small B lymphocytes, plasmacytoid lymphocytes and plasma cells. The symptoms of WM are protean, often follow an asymptomatic phase and may include complications related to the paraneoplastic effects of IgM paraprotein. The revised 2016 World Health Organization classification includes the MYD88 L265P mutation, which is seen in >90% of cases, within the diagnostic criteria for WM...
January 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28076302/detecting-paraprotein-interference-on-a-direct-bilirubin-assay-by-reviewing-the-photometric-reaction-data
#4
Elena García-González, Maite Aramendía, Ricardo González-Tarancón, Naiara Romero-Sánchez, Luis Rello
BACKGROUND: The direct bilirubin (D-Bil) assay on the AU Beckman Coulter instrumentation can be interfered by paraproteins, which may result in spurious D-Bil results. In a previous work, we took advantage of this fact to detect this interference, thus helping with the identification of patients with unsuspected monoclonal gammopathies. In this work, we investigate the possibility to detect interference based on the review of the photometric reactions, regardless of the D-Bil result. METHODS: The D-Bil assay was carried out in a set of 2164 samples...
January 11, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28068803/annals-express-artifactual-25-oh-vitamin-d-level-in-multiple-myeloma
#5
Michal Wen Sheue Ong, Rashim Salota, Tracy Reeman, Lapsley Marta, Lydia Jones
The most commonly used techniques to measure vitamin D are automated immunoassays which are known to be affected by interferences, especially from immunoglobulins present in the patient's serum. We present a case of a patient with myeloma in whom interference with the vitamin D assay was identified. An eighty-three year old female, known to have IgG myeloma was found to have a high concentration of 25-OH vitamin D on a routine test without any signs of vitamin D toxicity. She was not taking vitamin D supplements or any other multivitamin preparation and had minimal sun exposure...
January 1, 2017: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/27994856/c3-glomerulopathy-in-adults-a-distinct-patient-subset-showing-frequent-association-with-monoclonal-gammopathy-and-poor-renal-outcome
#6
Isaac E Lloyd, Alexander Gallan, Hunter K Huston, Kalani L Raphael, Dylan V Miller, Monica P Revelo, Mazdak A Khalighi
BACKGROUND: C3 glomerulopathy (C3G) includes both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD) and is defined by C3-dominant deposits on immunofluorescence. Dysfunction of the alternative pathway (AP) of complement is central to the pathophysiology of C3G and young patients often harbor genetic alterations of AP mediators. Recently, a link between C3G and paraproteinemia has been established. We performed this study to better characterize older patients with C3G where this association is more frequently seen...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27957423/crystalline-podocytopathy-and-tubulopathy-without-overt-glomerular-proteinuria-in-a-patient-with-multiple-myeloma
#7
Eun Jeong Lee, Su Yeon Lee, So Young Park, Yonjin Kim, Jae Shin Choi, Mi Jeoung Kim, Ji Hyeon Park, Jung Eun Lee, Ghee Young Kwon, Yoon-Goo Kim
Crystalline nephropathy is a rare yet well-known condition associated with multiple myeloma and other light chain-secreting disorders. Paraproteins that are resistant to proteolysis crystallize within proximal tubular cells and cause light-chain proximal tubulopathy, which presents clinically as Fanconi syndrome. Podocytes are rarely affected, and the crystalline inclusions within podocytes are typically precipitated, yielding significant glomerular proteinuria. Here we report a case of extensive crystalline inclusions primarily within podocytes and proximal tubules that presented only with Fanconi syndrome and renal insufficiency...
December 2016: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/27927893/paraprotein-related-kidney-disease-attack-of-the-killer-m-proteins
#8
Mark A Perazella, Kevin W Finkel
Paraproteins are monoclonal Igs or their components (light or heavy chains) that are produced by a clonal population of mature B cells, most commonly plasma cells. These paraproteins or monoclonal proteins are secreted into the blood and subsequently filtered by the glomerulus before entering into urine, where they can cause various types of kidney disease, including both glomerular and tubulointerstitial injuries. Furthermore, a monoclonal protein that causes a specific glomerular or tubulointerstitial lesion in a human can reproducibly cause the same pathology when injected into an animal, supporting unique paraprotein characteristics...
December 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27868410/-expression-of-microrna-221-222-in-patients-with-monoclonal-gammopathy-of-undetermined-significance-and-multiple-myeloma
#9
Suwen Yang, Wei Wang, Hong Jin, Yuhong Zhong, Xinyou Xie
Objective: To detect the expression of miR-221/222 in serum and plasma cells in patients with monoclonal gammopathy of undetermined significance(MGUS) and multiple myeloma(MM), and to explore the possibility of miR-221/222 as biomarkers in the diagnosis and prognosis predicting of MGUS and MM. Methods: Bone marrow and serum samples from 14 patients with newly diagnosed MGUS, 81 patients with newly diagnosed or relapsed MM and 10 controls were collected from Sir Run Run Shaw Hospital of Zhejiang University and Tongde Hospital of Zhejiang Province during January 2013 and December 2015...
May 25, 2016: Zhejiang da Xue Xue Bao. Yi Xue Ban, Journal of Zhejiang University. Medical Sciences
https://www.readbyqxmd.com/read/27866585/plasma-cell-disorders
#10
REVIEW
Jorge J Castillo
Plasma cell disorders are benign, premalignant, and malignant conditions characterized by the presence of a monoclonal paraprotein detected in serum or urine. These conditions are biologically, pathologically, and clinically heterogeneous. There have been major advances in the understanding of the biology of these diseases, which are promoting the development of therapies with novel mechanisms of action. Novel agents such as proteasome inhibitors, immunomodulatory drugs, and monoclonal antibodies have gained approval in the United States and Europe for the treatment of plasma cell disorders...
December 2016: Primary Care
https://www.readbyqxmd.com/read/27804161/monoclonal-gammopathy-of-undetermined-significance-mgus-and-smoldering-myeloma-smm-a-practical-guide-to-management
#11
Nicola Maciocia, Ashutosh Wechalekar, Kwee Yong
Monoclonal gammopathy of undetermined significance and smoldering multiple myeloma are precursor conditions of symptomatic multiple myeloma (MM). Diagnostic principles are aimed at excluding MM requiring therapy, other conditions associated with paraproteins that may require different management, and risk stratifying patients for the purposes of tailored follow-up and investigation. The International Myeloma Working Group have recently published a revised definition of MM, which singles out a small group of patients with smoldering multiple myeloma who are at very high risk of progression and organ damage; such patients are now included under the definition of MM and recommended to start anti-myeloma treatment...
November 2, 2016: Hematological Oncology
https://www.readbyqxmd.com/read/27775221/light-chain-podocytopathy-mimicking-recurrent-focal-segmental-glomerulosclerosis
#12
M A Khalighi, M P Revelo, J D Abraham, F Shihab, F Ahmed
Kidney injury related to paraproteinemia is common and typically occurs after the fourth decade of life in association with an underlying plasma cell dyscrasia or other lymphoproliferative disease. Kidney transplantation in paraprotein-related kidney disease can be successful in conjunction with treatment of the underlying hematopoietic process; however, when hematologic response to therapy is not achieved, recurrent kidney injury is frequently seen. We describe a young male patient who presented at the age of 23 years with end-stage kidney disease thought to be secondary to focal segmental glomerulosclerosis; this patient ultimately received two kidney allografts...
October 24, 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/27772927/immunotactoid-glomerulopathy-with-igm-positivity-and-associated-igm-paraprotein
#13
T Phillips, M Brown, M E Koina
No abstract text is available yet for this article.
February 2016: Pathology
https://www.readbyqxmd.com/read/27701752/immunotherapy-for-igm-anti-myelin-associated-glycoprotein-paraprotein-associated-peripheral-neuropathies
#14
REVIEW
Michael Pt Lunn, Eduardo Nobile-Orazio
BACKGROUND: Serum monoclonal anti-myelin-associated glycoprotein (anti-MAG) antibodies may be pathogenic in some people with immunoglobulin M (IgM) paraprotein and demyelinating neuropathy. Immunotherapies aimed at reducing the level of these antibodies might be expected to be beneficial. This is an update of a review first published in 2003 and previously updated in 2006 and 2012. OBJECTIVES: To assess the effects of immunotherapy for IgM anti-MAG paraprotein-associated demyelinating peripheral neuropathy...
October 4, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27699053/lessons-from-treatment-resistant-hyperlipidaemia
#15
Elizabeth Parsons, Pankaj Gupta, Prashanth Patel, Faizanur Rahman
A 68-year-old woman was referred to Lipid Clinic with sudden deterioration of previously well-controlled primary hyperlipidaemia. Investigations revealed nephrotic range proteinuria, leading to urgent renal biopsy and a diagnosis of amyloidosis. Chemotherapy was successful in stabilising renal function, reducing proteinuria and eliminating serum paraprotein. The resistant hyperlipidaemia subsequently resolved. Whilst hyperlipidaemia is pathognomonic of nephrotic syndrome, it is rarely the first characteristic identified by clinicians, often preceded by the identification of oedema or proteinuria...
October 2016: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/27590107/a-phase-i-ii-study-of-escalating-doses-of-bortezomib-in-conjunction-with-high-dose-melphalan-as-a-conditioning-regimen-for-salvage-autologous-peripheral-blood-stem-cell-transplantation-in-patients-with-multiple-myeloma
#16
Noa Biran, Scott D Rowley, David H Vesole, Shijia Zhang, Michele L Donato, Joshua Richter, Alan P Skarbnik, Andrew Pecora, David S Siegel
Escalating doses of bortezomib with high-dose melphalan was evaluated as as a conditioning regimen for autologous stem cell transplantation (ASCT) in patients with relapsed or refractory multiple myeloma (MM). MM patients with less than a partial remission (PR) (or 50% reduction) compared to their pretransplantation paraprotein parameters after a prior ASCT with melphalan conditioning, or who were in relapse after a prior autologous transplantation, were eligible for study. Bortezomib was dose escalated in steps of 1, 1...
August 31, 2016: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/27526708/paraprotein-related-kidney-disease-evaluation-and-treatment-of-myeloma-cast-nephropathy
#17
Kevin W Finkel, Eric P Cohen, Anushree Shirali, Ala Abudayyeh
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 is caused by the interaction and aggregation of filtered free light chains and Tamm-Horsfall protein causing intratubular obstruction and damage. The key to treating cast nephropathy is rapid lowering of free light chains, because this correlates with renal recovery...
December 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27526707/paraprotein-related-kidney-disease-kidney-injury-from-paraproteins-what-determines-the-site-of-injury
#18
Mona Doshi, Amit Lahoti, Farhad R Danesh, Vecihi Batuman, Paul W Sanders
Disorders of plasma and B cells leading to paraproteinemias are associated with a variety of renal diseases. Understanding the mechanisms of injury and associated nephropathies provides a framework that aids clinicians in prompt diagnosis and appropriate adjunctive treatment of these disorders. Glomerular diseases that may be associated with paraproteinemias include amyloid deposition, monoclonal Ig deposition disease, proliferative GN with monoclonal Ig deposits, C3 glomerulopathy caused by alterations in the complement pathway, immunotactoid glomerulopathy, fibrillary GN, and cryoglobulinemia...
December 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27526706/paraprotein-related-kidney-disease-glomerular-diseases-associated-with-paraproteinemias
#19
Shveta S Motwani, Leal Herlitz, Divya Monga, Kenar D Jhaveri, Albert Q Lam
Paraproteins are monoclonal Igs that accumulate in blood as a result of abnormal excess production. These circulating proteins cause a diversity of kidney disorders that are increasingly being comanaged by nephrologists. In this review, we discuss paraprotein-related diseases that affect the glomerulus. We provide a broad overview of diseases characterized by nonorganized deposits, such as monoclonal Ig deposition disease (MIDD), proliferative GN with monoclonal Ig deposits (PGNMID), and C3 glomerulopathy, as well as those characterized by organized deposits, such as amyloidosis, immunotactoid glomerulopathy, fibrillary GN, and cryoglobulinemic GN, and rarer disorders, such as monoclonal crystalline glomerulopathies, paraprotein-related thrombotic microangiopathies, and membranous-like glomerulopathy with masked IgGκ deposits...
December 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27526705/paraprotein-related-kidney-disease-diagnosing-and-treating-monoclonal-gammopathy-of-renal-significance
#20
Mitchell H Rosner, Amaka Edeani, Motoko Yanagita, Ilya G Glezerman, Nelson Leung
Paraprotein-related kidney disease represents a complex group of diseases caused by an abnormal paraprotein secreted by a clone of B cells. The disease manifestations range from tubulopathies, such as the Fanconi syndrome, to a spectrum of glomerular diseases that can present with varying degrees of proteinuria and renal dysfunction. Diagnosis of these diseases can be challenging because of the wide range of manifestations as well as the relatively common finding of a serum paraprotein, especially in elderly patients...
December 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
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