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https://www.readbyqxmd.com/read/29759418/a-clone-directed-approach-may-improve-diagnosis-and-treatment-of-proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-deposits
#1
Ramnika Gumber, Jordana B Cohen, Matthew B Palmer, Sidney M Kobrin, Dan T Vogl, Alan G Wasserstein, Sunita D Nasta, Melissa B Bleicher, Roy D Bloom, Laura Dember, Adam Cohen, Brendan M Weiss, Jonathan J Hogan
The optimal treatment for the monoclonal gammopathies of renal significance is not known, but there is consensus among experts that treatment should be specific for the underlying clone. The majority of patients with proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) do not have an identifiable clone, and prior studies have found poor renal outcomes for patients with PGNMID treated with a variety of regimens. Here we present a retrospective case series of 19 patients with PGNMID with a more uniform, clone-directed approach...
May 11, 2018: Kidney International
https://www.readbyqxmd.com/read/29743095/roxithromycin-monotherapy-inducing-a-partial-response-in-a-patient-with-myeloma-a-case-report
#2
Kern Y Chai, Anna L Byrne, Ian M Morison
BACKGROUND: Clarithromycin is an efficacious treatment for myeloma in combination with other anti-myeloma therapy but not as monotherapy. To date, all studies have focused on a clarithromycin-specific effect rather than a class effect (macrolide) and there is no information on the activity of roxithromycin in myeloma. CASE PRESENTATION: Here we report an untreated 86-year-old New Zealand European white man with IgA myeloma whose paraprotein decreased by 57%, consistent with a partial response, after a course of roxithromycin for pneumonia...
May 10, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29716794/proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-g-deposits-is-associated-with-high-rate-of-early-recurrence-in-the-allograft
#3
Samar M Said, Fernando G Cosio, Anthony M Valeri, Nelson Leung, Sanjeev Sethi, Hassan Salameh, Lynn D Cornell, Mary E Fidler, Mariam P Alexander, Fernando C Fervenza, Maria Eleni Drosou, Da Zhang, Vivette D D'Agati, Samih H Nasr
The characteristics of allograft proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) are not well defined. To better characterize this disease we retrospectively identified 26 patients with allograft PGNMID, including 16 followed with early protocol biopsies. PGNMID was found to be a recurrent disease in most (89%) patients. A diagnostic biopsy was done for proteinuria and/or increased creatinine in most patients. Median time from transplant to diagnostic biopsy was 5.5 months, with detection within three to four months post-transplant in 86% of patients...
April 28, 2018: Kidney International
https://www.readbyqxmd.com/read/29559475/germline-lysine-specific-demethylase-1-lsd1-kdm1a-mutations-confer-susceptibility-to-multiple-myeloma
#4
Xiaomu Wei, M Nieves Calvo-Vidal, Siwei Chen, Gang Wu, Maria V Revuelta, Jian Sun, Jinghui Zhang, Michael F Walsh, Kim E Nichols, Vijai Joseph, Carrie Snyder, Celine M Vachon, James D McKay, Shu-Ping Wang, David S Jayabalan, Lauren M Jacobs, Dina Becirovic, Rosalie G Waller, Mykyta Artomov, Agnes Viale, Jayeshkumar Patel, Jude Phillip, Selina Chen-Kiang, Karen Curtin, Mohamed Salama, Djordje Atanackovic, Ruben Niesvizky, Ola Landgren, Susan L Slager, Lucy A Godley, Jane Churpek, Judy E Garber, Kenneth C Anderson, Mark J Daly, Robert G Roeder, Charles Dumontet, Henry T Lynch, Charles G Mullighan, Nicola J Camp, Kenneth Offit, Robert J Klein, Haiyuan Yu, Leandro Cerchietti, Steven M Lipkin
Given the frequent and largely incurable occurrence of multiple myeloma, identification of germline genetic mutations that predispose cells to multiple myeloma may provide insight into disease etiology and the developmental mechanisms of its cell of origin, the plasma cell (PC). Here, we identified familial and early-onset multiple myeloma kindreds with truncating mutations in lysine-specific demethylase 1 (LSD1/KDM1A), an epigenetic transcriptional repressor that primarily demethylates histone H3 on lysine 4 and regulates hematopoietic stem cell self-renewal...
May 15, 2018: Cancer Research
https://www.readbyqxmd.com/read/29556454/surgical-thyroparathyroidectomy-prevents-progression-of-5tgm1-murine-multiple-myeloma-in-vivo
#5
REVIEW
Maurizio Zangari, Hanna Yoo, Ik Jae Shin, Donghoon Yoon, Larry J Suva
The 5TGM1 multiple myeloma transplanted C57BL6/KaLwRij model recapitulates many disease features including monoclonal paraprotein production as well as the development of osteolytic bone lesions. Since a significant association between serum parathyroid hormone PTH variations, bone anabolism and myeloma progression in patients receiving proteasome inhibitors exists, this study investigated the effect of the PTH axis on murine myeloma development in vivo . C57BL6/KaLwRij myeloma-bearing mice underwent thyroparathyroidectomy (TPTX) before and after 5TGM1 cell transplantation...
September 2018: Journal of Bone Oncology
https://www.readbyqxmd.com/read/29492600/lymphoma-like-monoclonal-b-cell-lymphocytosis-in-a-patient-population-biology-natural-evolution-and-differences-from-cll-like-clones
#6
Sam Vander Meeren, Bert Heyrman, Wim Renmans, Marleen Bakkus, Brigitte Maes, Hendrik De Raeve, Rik Schots, Kristin Jochmans
High-count monoclonal B cell lymphocytosis (MBL) with a chronic lymphocytic leukemia (CLL) phenotype is a well-known entity, featuring 1-4% annual risk of progression towards CLL requiring treatment. Lymphoma-like MBL (L-MBL), on the other hand, remains poorly defined and data regarding outcome are lacking. We retrospectively evaluated 33 L-MBL cases within our hospital population and compared them to 95 subjects with CLL-like MBL (C-MBL). Diagnoses of L-MBL were based on asymptomatic B cell clones with Matutes score < 3, B cells < 5...
February 28, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29485793/multiple-myeloma-with-advanced-bone-disease-and-low-tumor-burden-different-clinical-presentation-but-similar-outcome-after-bortezomib-based-therapy-and-radiotherapy
#7
Vesselina Goranova-Marinova, Marianna Yaneva, Tanya Deneva, Stefan Goranov, Dubravka Mužina Mišić
There is a small but well recognized group of patients with multiple myeloma (MM), characterized by multiple bone lesions and low tumor burden, the so-called macrofocal form of MM (MF-MM). The aim of the study was to analyze the incidence, clinical manifestation, therapeutic outcome and prognosis of patients with MF-MM treated with bortezomib-based therapy and radiotherapy, in comparison to classic MM. There were 148 MM patients treated with bortezomibbased regimens, with 15 (10.1%) of them meeting the criteria for MF-MM...
June 2017: Acta Clinica Croatica
https://www.readbyqxmd.com/read/29484184/endoplasmic-reticulum-stress-in-the-development-of-multiple-myeloma-and-drug-resistance
#8
REVIEW
Nicholas Nikesitch, James M Lee, Silvia Ling, Tara Laurine Roberts
Multiple myeloma (MM) is a haematological malignancy of mature antibody-secreting plasma cells. Currently, MM is incurable, but advances in drug treatments have increased patient lifespan. One of the characteristics of MM is the excessive production of monoclonal immunoglobulin (also referred to as paraprotein). This high level of protein production induces endoplasmic reticulum (ER) stress, and proteasomal degradation of the paraprotein is required to avoid ER stress-induced cell death. Consequently, proteasomal inhibitors such as bortezomib have been particularly effective therapies...
2018: Clinical & Translational Immunology
https://www.readbyqxmd.com/read/29477943/the-use-of-emergency-apheresis-in-the-management-of-plasma-cell-disorders
#9
REVIEW
Sevgi Kalayoglu-Besisik
Hyperviscosity syndrome (HVS) develops most commonly in Waldenström's macroglobulinemia (WM) and multiple myeloma (MM). Plasmapheresis is the immediate therapy and very effective at relieving symptoms by removing paraprotein. The most commonly used replacement fluid is 4%-5% human albumin in physiologic saline. FFP may be used in patients with coagulation abnormalities. Plasmapheresis should be continued until acute symptoms abate. Hyperviscosity impairs the circulation in the retina and causes hemorrhages around the small retinal vessels...
February 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29472805/gamma-heavy-chain-disease-accompanied-with-follicular-lymphoma-a-case-report
#10
Paula San-José, Vicente Aguadero, Granada Perea, Meritxell Estrada, Eugenio Berlanga
Heavy chain diseases (HCD) are B-cell lymphoprolipherative disorders characterized by the production of monoclonal heavy chains without associated light chains. Some cases of gamma-HCD (γ-HCD) are concurrent with other lymphoid neoplasm. The monoclonal component is not always detectable by serum electrophoresis, and often an immunofixation procedure is necessary to detect this component. Prognosis is variable, and no established guidelines for follow-up are available. We describe a case of a challenging diagnosis of γ-HCD due to the absence of clinical signs frequently reported in the disease (anaemia and palatal oedema among others)...
February 15, 2018: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
https://www.readbyqxmd.com/read/29429912/denosumab-versus-zoledronic-acid-in-bone-disease-treatment-of-newly-diagnosed-multiple-myeloma-an-international-double-blind-double-dummy-randomised-controlled-phase-3-study
#11
Noopur Raje, Evangelos Terpos, Wolfgang Willenbacher, Kazuyuki Shimizu, Ramón García-Sanz, Brian Durie, Wojciech Legieć, Marta Krejčí, Kamel Laribi, Li Zhu, Paul Cheng, Douglas Warner, G David Roodman
BACKGROUND: Multiple myeloma is characterised by monoclonal paraprotein production and osteolytic lesions, commonly leading to skeletal-related events (spinal cord compression, pathological fracture, or surgery or radiotherapy to affected bone). Denosumab, a monoclonal antibody targeting RANKL, reduces skeletal-related events associated with bone lesions or metastases in patients with advanced solid tumours. This study aimed to assess the efficacy and safety of denosumab compared with zoledronic acid for the prevention of skeletal-related events in patients with newly diagnosed multiple myeloma...
March 2018: Lancet Oncology
https://www.readbyqxmd.com/read/29386839/intensely-pruritic-papules-and-plaques-in-waldenstrom-s-macroglobulinemia
#12
Solam Lee, Noo Ri Lee, Sung Jay Choe, Beom Jun Kim, Minseob Eom, Eung Ho Choi
Waldenstrom's macroglobulinemia (WM) is lymphoplasmacytoid malignancy that affects B lymphocytes. Cutaneous involvement of WM is rare, but various cutaneous manifestations have been reported. These findings are due to various pathological processes including direct invasion of tumor cells into the skin, deposition of paraproteins, hyperviscosity syndrome, and cryoglobulinemia. A 64-year-old man presented with a 10-day history of pruritic erythematous papules and plaques on his trunk and elbows. The clinical features were suspicious for eczematous dermatitis...
February 2018: Annals of Dermatology
https://www.readbyqxmd.com/read/29367362/primary-meningococcal-arthritis-as-a-presentation-of-nodal-marginal-zone-lymphoma
#13
Gemma Ann Joan Harrop, Jane Tighe, Alexander MacKenzie
A 68-year-old man presented with a 4-day history of worsening knee and arm pain. On examination, there was erythema and swelling of the left knee and both wrists. Joint aspiration grew Neisseria meningitidis Blood tests showed an unusually high total protein level (100 g/L) and an IgM kappa paraprotein band of 45 g/L on protein electrophoresis. CT showed widespread lymphadenopathy, hepatosplenomegaly and multilevel thoracic vertebral collapse. A bone marrow biopsy revealed a lymphoplasmacytic infiltrate and a lymph node biopsy showed features of nodal marginal zone lymphoma with plasmacytic differentiation...
January 23, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29360169/adrenomedullin-surges-are-linked-to-acute-episodes-of-the-systemic-capillary-leak-syndrome-clarkson-disease
#14
Zhihui Xie, Wei-Sheng Chen, Yuzhi Yin, Eunice C Chan, Kaoru Terai, Lauren M Long, Timothy G Myers, Arkadiusz Z Dudek, Kirk M Druey
BACKGROUND: Systemic Capillary Leak Syndrome (SCLS) is an extremely rare and life-threatening vascular disorder of unknown etiology. SCLS is characterized by abrupt and transient episodes of hypotensive shock and edema due to plasma leakage into peripheral tissues. The disorder has garnered attention recently because its initial presentation resembles more common vascular disorders including systemic anaphylaxis, sepsis, and acute infections with the Ebola/Marburg family of filoviruses...
April 2018: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29315287/monitoring-multiple-myeloma
#15
Kyle A Udd, Tanya M Spektor, James R Berenson
Multiple myeloma (MM) is a B-cell malignancy characterized by the accumulation in bone marrow of terminally differentiated plasma cells. MM is a slowly growing, heterogeneous disease with no known cure. Patients with MM have a median survival of approximately 5 years, during which they may experience significant morbidity. More reliable and rapid determination of changes in the clinical status of patients with MM is becoming increasingly important because of the increasing number of available treatments for these patients...
December 2017: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29290952/transient-paraproteinemia-after-allogeneic-hematopoietic-stem-cell-transplantation-is-an-underexplored-phenomenon-associated-with-graft-versus-host-disease
#16
Corinne C Widmer, Stefan Balabanov, Urs Schanz, Alexandre P A Theocharides
The clinical and biological relevance of a paraprotein that newly arises after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in non-myeloma patients is unknown. In this study, the incidence, the course, and the clinical impact of paraproteins found after allo-HSCT were investigated in a cohort of 383 non-myeloma patients. Paraproteinemia after allo-HSCT was more frequent (52/383 patients, 14%) than the reported incidence of monoclonal gammopathy of unknown significance (MGUS) in age-matched healthy subjects and, in contrast to MGUS, did not correlate with age...
December 5, 2017: Oncotarget
https://www.readbyqxmd.com/read/29284595/molecular-genetic-investigation-clinical-features-and-response-to-treatment-in-21-patients-with-schnitzler-syndrome
#17
Dorota M Rowczenio, Shelly Pathak, Juan I Arostegui, Anna Mensa-Vilaro, Ebun Omoyinmi, Paul Brogan, Dan Lipsker, Thomas Scambler, Roger Owen, Hadija Trojer, Anna Baginska, Julian D Gillmore, Ashutosh D Wechalekar, Thirusha Lane, Rene Williams, Taryn Youngstein, Philip N Hawkins, Sinisa Savic, Helen J Lachmann
To date, the pathogenic mechanisms underlying Schnitzler syndrome remain obscure, in particular, the interplay between the monoclonal protein and increased interleukin-1β (IL-1β) production, although interest in the contribution of genetic factors has been fueled by detection of somatic NLRP3 mosaicism in 2 patients with the variant-type Schnitzler syndrome. At 2 specialist UK centers, we have identified 21 patients who fulfilled diagnostic criteria for Schnitzler syndrome with urticarial rash, fever, arthralgia, and bone pain; 47% reported weight loss, 40% fatigue, and 21% lymphadenopathy...
March 1, 2018: Blood
https://www.readbyqxmd.com/read/29281132/monoclonal-gammopathies-electronic-subspecialty-consultation
#18
Nicholas Burwick, Jacob Stein, David A Garcia, Virginia C Broudy, Robert E Richard
IMPORTANCE: Electronic consultation (e-consult) is an important component of care for patients in the Veterans Health Administration who require subspecialty consultation but not urgent face-to-face evaluation. Monoclonal gammopathy of undetermined significance (MGUS) is a common reason for e-consult. While often benign, MGUS requires careful evaluation and persistent surveillance over time. OBJECTIVE: To identify areas to improve MGUS care delivery by e-consult...
April 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29279596/light-chain-multiple-myeloma-an-evaluation-of-its-biochemical-investigations
#19
S Y Zahari Sham, S C Thambiah, I N Samsudin, S M Lim
Multiple myeloma is a type of plasma cell dyscrasia, characterised by presence of paraprotein or monoclonal (M)-protein in serum or urine. The M-protein may consist of an intact immunoglobulin, the heavy chain only or the light chain only. The latter, designated as light chain multiple myeloma (LCMM) makes up almost 20% of myelomas. Clinical manifestation is often heralded by hypercalcaemia, renal impairment, normocytic normochromic anaemia and bone lesions, reflecting end-organ damage, collectively known as the acronym CRAB...
December 2017: Malaysian Journal of Pathology
https://www.readbyqxmd.com/read/29230710/a-case-report-of-proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-m-kappa-deposits-without-associated-lymphoproliferative-disorder-or-detectable-paraproteinemia
#20
Yoshito Yamaguchi, Kunihiko Maeda, Katsuyuki Nagatoya, Atsushi Yamauchi
A 53-year-old man presented with proteinuria and hematuria. No significant abnormality was detected in his physical examination or laboratory tests, including evidence of paraprotein in serum and urine. Renal biopsy revealed mesangial proliferation, thickened glomerular basement membranes, and spike formation. Immunofluorescence revealed deposition of immunoglobulin (Ig) M heavy chain, kappa (κ) light chain, and complement component C3 along capillary walls in the glomeruli. Light chain staining indicated significant restriction, because only κ chain, not lambda chain, was present in glomeruli...
December 11, 2017: CEN Case Reports
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