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Plasma cell dyscrasias

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https://www.readbyqxmd.com/read/29409882/the-efficacy-and-long-term-outcomes-of-autologous-stem-cell-transplantation-in-poems-syndrome-a-nation-wide-survey-in-japan
#1
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, VEGF 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 TRUMP database...
January 31, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29398791/plasma-cell-dyscrasias-in-india-2017-updates
#2
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%C3%A2-review-of-the-literature%C3%A2
#3
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
#4
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)...
January 30, 2018: Hematological Oncology
https://www.readbyqxmd.com/read/29372057/right-orbital-edema-masquerading-a-hematologic-malignancy
#5
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
#6
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
#7
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
#8
Sarika Gupta, Nitin J Karandikar, Timothy Ginader, Andrew M Bellizzi, Carol J Holman
BACKGROUND: Multiparametric flow cytometry (MFC) is a useful tool for diagnosis of plasma cell dyscrasias and assessment of minimal residual disease (MRD) in plasma cell myeloma (PCM). However, the immunophenotypic differences between the clonal plasma cells (PCs) of plasma cell myeloma (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
#9
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/29209044/biological-and-prognostic-impact-of-apobec-induced-mutations-in-the-spectrum-of-plasma-cell-dyscrasias-and-multiple-myeloma-cell-lines
#10
F Maura, M Petljak, M Lionetti, I Cifola, W Liang, E Pinatel, L Alexandrov, A Fullam, I Martincorena, K J Dawson, N Angelopoulos, M K Samur, R Szalat, J Zamora, P Tarpey, H Davies, P Corradini, K Anderson, S Minvielle, A Neri, H Avet-Loiseau, J J Keats, P J Campbell, N Munshi, N Bolli
Leukemia accepted article preview online, 06 December 2017. doi:10.1038/leu.2017.345.
December 6, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29199718/multiple-myeloma-experience-of-an-institute-in-limited-resource-setting
#11
Linu Abraham Jacob, M C Suresh Babu, K C Lakshmaiah, K Govind Babu, D Lokanatha, L K Rajeev, K N Lokesh, A H Rudresha, Ankit Agarwal, Sunny Garg
INTRODUCTION: Multiple myeloma (MM) is a plasma cell dyscrasias and an incurable clonal B-cell malignancy, with an annual incidence of 1% of all malignancies. The mainstay of treatment of myeloma is induction treatment followed by consolidation with autologous stem cell transplant (ASCT). However, still in a developing country like India where affordability is a major hurdle for health care, a number of MM patients are not able to undergo ASCT. AIM: To study the epidemiological features and outcome of MM patients treated in a limited resource setting...
January 2017: Indian Journal of Cancer
https://www.readbyqxmd.com/read/29184450/coexisting-multiple-myeloma-lymphoma-and-non-small-cell-lung-cancer-a-case-report-and-review-of-the-literature
#12
Parth Khade, Srinivas Devarakonda
Multiple myeloma is a plasma cell dyscrasia characterized by neoplastic proliferation of plasma cells, producing a monoclonal immunoglobulin. Small lymphocytic lymphoma (SLL) is a neoplasm consisting of monoclonal B-cell lymphocyte proliferation. We present an extremely rare case of coexisting multiple myeloma, SLL, and squamous cell carcinoma of the lung in a 74-year-old female patient. She initially presented with a midline mass with pain in the lumbar area. Debulking surgery was performed, and pathology showed plasmacytoma...
2017: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/29176541/diffuse-peritoneal-and-bowel-wall-infiltration-by-light-chain-al-amyloidosis-with-omental-calcification-mimicking-abdominal-carcinomatosis-an-elderly-female-with-incidental-finding-of-light-chain-monoclonal-gammopathy-of-undetermined-significance-lc-mgus
#13
Shoaib Junejo, Yasir Ali, Sandeep Singh Lubana, Sandeep S Tuli
BACKGROUND Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type. There have been no reports of obstructive GIT AL amyloid patients having monoclonal gammopathy of undetermined significance (MGUS)...
November 25, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29166521/case-for-diagnosis-systemic-light-chain-amyloidosis-with-cutaneous-involvement
#14
João Renato Vianna Gontijo, Jackson Machado Pinto, Maysa Carla de Paula
Systemic light chain amiloydosis is a rare disease. Due to its typical cutaneous lesions, dermatologists play an essential role in its diagnosis. Clinical manifestations vary according to the affected organ and are often unspecific. Definitive diagnosis is achieved through biopsy. We report a patient with palpebral amyloidosis, typical bilateral ecchymoses and cardiac involvement, without plasma cell dyscrasia or lymphomas. The patient died shortly after the diagnosis.
September 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29159010/multiple-myeloma-presenting-as-acute-renal-failure-in-the-absence-of-other-characteristic-features
#15
Zachary N Gastelum, Diana M Biggs, Aaron Scott
This case report describes a 54-year-old, asymptomatic man who presented with hyperkalemia on routine lab testing who was later found to have acute renal failure, unresponsive to fluid resuscitation, with minimal improvement after hemodialysis. After a comprehensive evaluation ruled out common causes of acute renal failure, the patient underwent testing with a bone survey, urine protein electrophoresis (UPEP), serum protein electrophoresis (SPEP), and immunoelectrophoresis for suspected plasma cell dyscrasia and received plasmapheresis for hyperviscosity syndrome and nephrotoxicity, which resulted in improved renal function...
September 20, 2017: Curēus
https://www.readbyqxmd.com/read/29157614/poems-syndrome-diagnosis-and-investigative-work-up
#16
REVIEW
Angela Dispenzieri, Taxiarchis Kourelis, Francis Buadi
POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease is the first step in managing the disease. Increased blood levels of vascular endothelial growth factor are usually confirmatory. This rare disorder should not be missed, especially if the patient has a putative diagnosis of chronic inflammatory polyradiculoneuropathy, a lambda restricted monoclonal gammopathy, and thrombocytosis, and is not responding as expected to immunomodulatory therapy commonly used for chronic inflammatory polyradiculoneuropathy...
February 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29142973/renal-amyloidosis-associated-with-5-novel%C3%A2-variants-in-the-fibrinogen-a-alpha-chain-protein
#17
Dorota Rowczenio, Maria Stensland, Gustavo A de Souza, Erik H Strøm, Janet A Gilbertson, Graham Taylor, Nigel Rendell, Shane Minogue, Yvonne A Efebera, Helen J Lachmann, Ashutosh D Wechalekar, Philip N Hawkins, Ketil R Heimdal, Kristian Selvig, Inger K Lægreid, Nathalie Demoulin, Selda Aydin, Julian D Gillmore, Tale N Wien
Introduction: Fibrinogen A alpha chain amyloidosis is an autosomal dominant disease associated with mutations in the fibrinogen A alpha chain (FGA) gene, and it is the most common cause of hereditary renal amyloidosis in the UK. Patients typically present with kidney impairment and progress to end-stage renal disease over a median time of 4.6 years. Methods: Six patients presented with proteinuria, hypertension, and/or lower limb edema and underwent detailed clinical and laboratory investigations...
May 2017: KI Reports
https://www.readbyqxmd.com/read/29128071/diagnosis-of-plasma-cell-dyscrasias-and-monitoring-of-minimal-residual-disease-by-multiparametric-flow-cytometry
#18
REVIEW
Kah Teong Soh, Joseph D Tario, Paul K Wallace
Plasma cell dyscrasia (PCD) is a heterogeneous disease that has seen a tremendous change in outcomes due to improved therapies. Over the past few decades, multiparametric flow cytometry has played an important role in the detection and monitoring of PCDs. Flow cytometry is a high-sensitivity assay for early detection of minimal residual disease (MRD) that correlates well with progression-free survival and overall survival. Before flow cytometry can be effectively implemented in the clinical setting, sample preparation, panel configuration, analysis, and gating strategies must be optimized to ensure accurate results...
December 2017: Clinics in Laboratory Medicine
https://www.readbyqxmd.com/read/29111449/editorial-on-laboratory-diagnosis-and-management-of-plasma-cell-dyscrasias-special-issue
#19
EDITORIAL
David Keren, Christopher R McCudden, Ronald A Booth
No abstract text is available yet for this article.
October 27, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/29107338/prevalence-and-clinicopathologic-characteristics-of-multiple-myeloma-with-cutaneous-involvement-a-case-series-from-korea
#20
Yu Ri Woo, Jong Sic Kim, Ji Hong Lim, Sewon Hwang, Miri Kim, Jung Min Bae, Young Min Park, Chang-Ki Min, Dong-Wook Kim, Hyun Jeong Park
BACKGROUND: Multiple myeloma (MM) is a plasma cell dyscrasia characterized by the presence of a clonal proliferation of tumor cells. Cutaneous involvement of MM is very rare and remains poorly understood. OBJECTIVE: The aim of this study was to examine the clinical and histopathologic characteristics of cutaneous involvement in MM and identify factors associated with overall survival of MM with cutaneous involvement. METHODS: The medical records of 1228 patients with MM were retrieved and analyzed...
October 26, 2017: Journal of the American Academy of Dermatology
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