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Plasma cell dyscrasia

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https://www.readbyqxmd.com/read/29767409/the-use-of-serum-free-light-chain-dimerization-patterns-assist-in-the-diagnosis-of-al-amyloidosis
#1
Moshe E Gatt, Batia Kaplan, Dean Yogev, Elana Slyusarevsky, Galina Pogrebijski, Sizilia Golderman, Olga Kukuy, Avi Livneh
The discrimination between benign and malignant forms of plasma cell dyscrasia (PCD) is often difficult. Free light chain monomer-dimer pattern analysis (FLC-MDPA) may assist in solving this dilemma and distinguish between AL amyloidosis and benign PCD. Serum samples of patients with AL amyloidosis and benign PCD were analysed in a blinded manner. Quantitative Western blotting was performed to estimate dimerization and clonality indices, and thereby determine the source of the tested samples, as derived either from benign or malignant PCD...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29739081/subclasses-of-monoclonal-type-i-immunoglobulin-g-cryoglobulins-report-on-two-distinct-cases-with-myeloma
#2
Tania Petersen, Sebastien Riviere, Stephanie Malbos, Yannick Chantran, Aicha Abbas, Francois Chasset, Arsene Mekinian, Jeremie Sellam, Laurent Garderet, Pierre Aucouturier
BACKGROUND: While different clinical manifestations of IgM and IgG monoclonal cryoglobulins have been demonstrated, little is known about the roles of IgG subclasses in the pathophysiology of these conditions. METHODS: In two cases of myeloma-associated monoclonal (type I) cryoglobulinemia with quite distinct clinical and biological features, serum samples were analyzed using an original IgG subclass-specific immunoblotting technique. RESULTS: The first case had painful arthritis of hands and feet, with skin purpura and a sharp decrease of complement C4 level, and the cryoglobulin was of IgG1 subclass...
April 1, 2018: Clinical Laboratory
https://www.readbyqxmd.com/read/29721253/neurofibromatosis-type-i-and-multiple-myeloma-coexistence-a-possible-link
#3
Fabrizio Accardi, Valentina Marchica, Cristina Mancini, Elena Maredi, Costantina Racano, Laura Notarfranchi, Davide Martorana, Paola Storti, Eugenia Martella, Benedetta Dalla Palma, Luisa Craviotto, Massimo De Filippo, Antonio Percesepe, Franco Aversa, Nicola Giuliani
The association between Neurofibromatosis type I (NF1) and multiple myeloma (MM), a plasma cell, dyscrasia is very rare. Here we put to the attention of the scientific community two new cases. The first one is a patient with active MM whereas the second with smoldering MM. Both patients present typical features of NF1 but skeletal alterations were present only in the second case including dysplasia, marked scoliosis and osteoporosis. MM osteolytic lesions were absent in both patients. In addition to the clinical diagnosis of NF1, a molecular testing for NF1 gene mutations has been performed finding that patient one was heterozygous for the c...
March 2, 2018: Hematology Reports
https://www.readbyqxmd.com/read/29703720/from-mgus-to-multiple-myeloma-a-paradigm-for-clonal-evolution-of-premalignant-cells
#4
REVIEW
Niels van Nieuwenhuijzen, Ingrid Spaan, Reinier Raymakers, Victor Peperzak
Multiple myeloma (MM) is a treatable, but incurable, malignancy of plasma cells (PC) in the bone marrow (BM). It represents the final stage in a continuum of PC dyscrasias and is consistently preceded by a premalignant phase termed monoclonal gammopathy of undetermined significance (MGUS). The existence of this well-defined premalignant phase provides the opportunity to study clonal evolution of a premalignant condition into overt cancer. Unraveling the mechanisms of malignant transformation of PC could enable early identification of MGUS patients at high risk of progression and may point to novel therapeutic targets, thereby possibly delaying or preventing malignant transformation...
April 27, 2018: Cancer Research
https://www.readbyqxmd.com/read/29660912/increased-plasma-viscosity-in-plasma-cell-dyscrasia-and-whole-blood-viscosity-in-polycythemia-vera
#5
Dong Woo Shin, Ja-Yoon Gu, Jun Sik Kim, Jae-Seol Jung, Dong-Yeop Shin, Youngil Koh, Inho Kim, Hyun Kyung Kim
BACKGROUND: Although hyperviscosity syndrome in plasma cell dyscrasia (PCD) and thrombosis in myeloproliferative neoplasm (MPN) are major causes of morbidity and mortality, blood viscosity measurements are often underutilized. OBJECTIVE: This study aimed to characterize whether whole blood viscosity (WBV) or plasma viscosity (PV) could be predictive of hyperviscosity syndrome in PCD and could be elevated in subgroups of MPN. METHODS: A total of 75 patients with hematologic diseases: PCD (n = 26), MPN (n = 25) including polycythemia vera (P...
April 13, 2018: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/29625928/serum-free-light-chain-difference-and-%C3%AE-2-microglobulin-levels-are-risk-factors-for-thromboembolic-events-in-patients-with-al-amyloidosis
#6
Hyunkyung Park, Ji-Won Kim, Jeonghwan Youk, Youngil Koh, Jeong-Ok Lee, Ki Hwan Kim, Soo-Mee Bang, Inho Kim, Seonyang Park, Sung-Soo Yoon
BACKGROUND: AL amyloidosis might increase the risk of thromboembolism and other plasma cell dyscrasias; however, only a few reports have described the clinical features of thromboembolism. The present study aimed to elucidate the clinical features of thromboembolic events and to identify the risk factors for these events. MATERIALS AND METHODS: The medical records were retrospectively reviewed to define the clinically significant thromboembolic events. RESULTS: A total of 106 patients with biopsy-proven AL amyloidosis were included...
March 15, 2018: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/29622867/autologous-stem-cell-transplantation-for-multiple-myeloma-single-centre-experience-from-north-india
#7
Pankaj Malhotra, Uday Yanamandra, Alka Khadwal, Gaurav Prakash, Deepesh Lad, Arjun D Law, Harshit Khurana, M U S Sachdeva, Praveen Bose, Reena Das, Neelam Varma, Subhash Varma
Autologous stem cell transplantation (ASCT) is considered as standard of care in patients with multiple myeloma (MM) patients aged 65 years or younger. We analyzed data of 94 patients of plasma cell dyscrasias who underwent 95 autologous transplants at our institute from October 2003 to Aug 2016. Other than 76 patients of newly diagnosed multiple myeloma, we also transplanted two patients of POEMS syndrome, two patients of plasma cell leukemia, three patients of concurrent light chain deposition disease, three patients of multifocal plasmacytomas, and eight patients of isolated light chain myeloma...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29606981/pleural-effusion-in-11-14-translocation-q1-multiple-myeloma-in-the-setting-of-proteasome-inhibitor-presents-therapeutic-complexity
#8
Malik Ghannam, Maria Bryan, Erik Kuross, Brent Berry
Background: Primary malignant pleural effusion has been reported in about 134 cases of multiple myeloma (MM). Associated pleural effusions in cases of MM portend a poor prognosis and identifying them is highly relevant. Reported is the case of a man diagnosed with MM who developed primary myelomatous pleural effusion in the setting of multiple relapses and subsequent mortality within 2 months of the pleural effusion diagnosis. Presentation: A 61-year-old African American man was diagnosed with MM in 2011...
2018: Memo
https://www.readbyqxmd.com/read/29589834/hierarchical-involvement-of-myeloid-derived-suppressor-cells-and-monocytes-expressing-latency-associated-peptide-in-plasma-cell-dyscrasias
#9
Tamar Tadmor, Ilana Levy, Zahava Vadasz
Plasma cell dyscrasias (PCD) are disorders of the plasma cells having in common the production of a monoclonal M-protein. They include a spectrum of conditions which may represent a natural progression of the same disease from monoclonal gammopathy of unknown significance (MGUS), to asymptomatic and symptomatic multiple myeloma and plasma cell leukemia and Waldenström's macroglobulinemia (WM). In PCD the immune system is actively suppressed through the secretion of suppressive factors and the recruitment of immune suppressive subpopulations...
March 28, 2018: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/29589647/primary-systemic-amyloidosis-with-skin-and-cardiac-involvement-a-case-report
#10
Anja Trajber Horvat, Katarina Trčko, Vesna Jurčić, Pij Bogomir Marko
Primary systemic amyloidosis is characterized by the deposition of insoluble monoclonal immunoglobulin light chains in various tissues and is usually associated with an underlying plasma cell dyscrasia. In the early stage of the disease, dermatological findings can be the only manifestation, as opposed to organ involvement in the later stages. A dermatologist can diagnose amyloidosis early with a skin biopsy stained with Congo red dye and other appropriate investigations. This case report describes a female patient with primary systemic amyloidosis confirmed histologically from a skin biopsy...
March 2018: Acta Dermatovenerologica Alpina, Panonica, et Adriatica
https://www.readbyqxmd.com/read/29472358/circulating-tumor-dna-as-a-liquid-biopsy-in-plasma-cell-dyscrasias
#11
Bernhard Gerber, Martina Manzoni, Valeria Spina, Alessio Bruscaggin, Marta Lionetti, Sonia Fabris, Marzia Barbieri, Gabriella Ciceri, Alessandra Pompa, Gabriela Forestieri, Erika Lerch, Paolo Servida, Francesco Bertoni, Emanuele Zucca, Michele Ghielmini, Agostino Cortelezzi, Franco Cavalli, Georg Stussi, Luca Baldini, Davide Rossi, Antonino Neri
No abstract text is available yet for this article.
February 22, 2018: Haematologica
https://www.readbyqxmd.com/read/29409882/efficacy-and-long-term-outcomes-of-autologous-stem-cell-transplantation-in-poems-syndrome-a-nationwide-survey-in-japan
#12
Chika Kawajiri-Manako, Emiko Sakaida, Chikako Ohwada, Toshihiro Miyamoto, Taichi Azuma, Jun Taguchi, Takehiko Mori, Yuichi Hasegawa, Tadakazu Kondo, Toshiaki Yujiri, Makoto Yoshimitsu, Kazunori Imada, Shingo Kurahashi, Kaoru Kahata, Tatsuo Ichinohe, Makoto Hirokawa, Yoshiko Atsuta, Chiaki Nakaseko
POEMS syndrome is a rare plasma cell dyscrasia presenting with polyneuropathy, λ-type M protein, vascular endothelial growth factor elevation, and systemic manifestations. The standard treatment has not been established, but autologous stem cell transplantation (ASCT) has exhibited effectiveness in this syndrome. However, the efficacy and long-term outcomes of ASCT have not been systematically studied. To clarify the efficacy and long-term outcomes of ASCT-treated patients in Japan, we performed a multicenter retrospective study assessing the clinical course of patients registered to the Japan Society for Hematopoietic Cell Transplantation Transplant Registry Unified Management Program (TRUMP) database...
February 1, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29398791/plasma-cell-dyscrasias-in-india-2017-updates
#13
Arihant Jain, Pankaj Malhotra
Rapid advances are being made in the field of plasma cell dyscrasias. Many abstracts pertaining to the laboratory aspects, clinical features, treatment modalities and outcome of plasma cell dyscrasias were presented at Hematocon 2017. All the total of 24 abstracts pertaining to plasma cell dyscrasias presented at the Hematocon 2017 were reviewed. Out of them 10 were original research and 14 were case reports/short case series. The key findings of original research studies conducted in India are being summarized...
January 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29393843/successful-treatment-of-nephrotic-syndrome-induced-by-lambda-light-chain-deposition-disease-using-lenalidomide-a-case-report-and-review-of-the-literature
#14
Akira Mima, Dai Nagahara, Kosuke Tansho
BACKGROUND: Light chain deposition disease (LCDD) is a monoclonal immunoglobulin deposition disease (MIDD) that is characterized by the deposition of monoclonal light chains in multiple organs, including the kidney. It is a rare disorder caused by an underlying monoclonal plasma cell dyscrasia. LCDD with renal involvement causes proteinuria, which sometimes can lead to nephrotic syndrome. The monoclonal light chains are mostly in the κ form. Treatment of LCDD is the same as that for multiple myeloma (MM); however, some conventional anticancer drugs show substantial toxicity and therefore cannot be administered to older patients or those with renal impairment...
February 2, 2018: Clinical Nephrology
https://www.readbyqxmd.com/read/29380910/chemical-and-thyroid-hormone-profile-of-the-bone-marrow-interstitial-fluid-in-hematologic-disorders-and-patients-without-primary-hematologic-disorders
#15
Eilon Krashin, Martin Ellis, Keren Cohen, Maya Viner, Eran Neumark, Gloria Rashid, Osnat Ashur-Fabian
Bone marrow interstitial fluid (BMIF) has not been well characterized. BMIF was isolated from 60 patients including plasma cell dyscrasias (PCD, n = 33), other primary hematologic disorders (OHD, n = 15), and patients with secondary or nonhemtologic disorders (NHD, n = 12) and analyzed for an array of chemical constituents. These included total cholesterol, glucose, phosphate, creatinine, urea, total protein, albumin, globulins, total bilirubin, aspartate aminotransferase, lactate dehydrogenase, sodium, osmolarity, free triiodothyronine (free T3), total triiodothyronine (total T3), and free tetraiodothyronine (free T4)...
April 2018: Hematological Oncology
https://www.readbyqxmd.com/read/29372057/right-orbital-edema-masquerading-a-hematologic-malignancy
#16
Andrew C Tiu, Vivian Arguello-Guerra, Gabor Varadi
Introduction: Multiple myeloma is caused by abnormal proliferation of plasma cells that affects more commonly African Americans. It classically presents with hypercalcemia, renal failure, anemia, and lytic bone lesions. The aim of this article is to present an unusual case of a 63-year-old African-American female with multiple myeloma who presented with worsening right-sided eye swelling for the past 3 weeks and to briefly review ophthalmologic manifestations of multiple myeloma. Case description: Our patient's presentation was associated with a throbbing frontal headache, nasal congestion, malaise, and weight loss...
2018: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29359290/-renal-involvement-in-amyloidosis-and-sarcoidosis
#17
Jörg Beimler, Martin Zeier
Amyloidosis is a rare disease characterized by extracellular deposition of fibrils. Among the most common forms of systemic amyloidosis with renal involvement are AL-amyloidosis based on plasma cell dyscrasia and AA-amyloidosis in chronic inflammatory diseases. Depending on the affected renal compartment, the clinical appearance of renal amyloidosis varies. The pattern of renal amyloid deposition can be glomerular, interstitial, tubular or even vascular. Renal amyloid deposits are detected by renal biopsy. Patients with glomerular deposits typically show severe nephrotic syndrome with volume overload...
January 2018: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29338789/diagnosis-treatment-and-response-assessment-in-solitary-plasmacytoma-updated-recommendations-from-a-european-expert-panel
#18
REVIEW
J Caers, B Paiva, E Zamagni, X Leleu, J Bladé, S Y Kristinsson, C Touzeau, N Abildgaard, E Terpos, R Heusschen, E Ocio, M Delforge, O Sezer, M Beksac, H Ludwig, G Merlini, P Moreau, S Zweegman, M Engelhardt, L Rosiñol
Solitary plasmacytoma is an infrequent form of plasma cell dyscrasia that presents as a single mass of monoclonal plasma cells, located either extramedullary or intraosseous. In some patients, a bone marrow aspiration can detect a low monoclonal plasma cell infiltration which indicates a high risk of early progression to an overt myeloma disease. Before treatment initiation, whole body positron emission tomography-computed tomography or magnetic resonance imaging should be performed to exclude the presence of additional malignant lesions...
January 16, 2018: Journal of Hematology & Oncology
https://www.readbyqxmd.com/read/29316245/flow-cytometric-aberrancies-in-plasma-cell-myeloma-and-mgus-correlation-with-laboratory-parameters
#19
Sarika Gupta, Nitin J Karandikar, Timothy Ginader, Andrew M Bellizzi, Carol J Holman
BACKGROUND: Multiparametric flow cytometry is a useful tool for diagnosis of plasma cell (PC) dyscrasias and assessment of minimal residual disease in plasma cell myeloma (PCM). However, the immunophenotypic differences between the clonal PCs of PCM and those of monoclonal gammopathy of undetermined significance (MGUS) as well as the correlation of these flow cytometric markers with pertinent laboratory parameters have not been evaluated. METHODS: We retrospectively identified all newly diagnosed treatment-naive PCM and MGUS patients between 09/2014 and 06/2015 who underwent 10-color flow-cytometric evaluation: CD45, CD38, CD138, cKappa, cLambda, CD19, CD27, CD28, CD56, CD117...
January 6, 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29279596/light-chain-multiple-myeloma-an-evaluation-of-its-biochemical-investigations
#20
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
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